DEFRA What is the risk of a cervid TSE being introduced from Norway into Great Britain? Qualitative Risk Assessment September 2016

DEFRA What is the risk of a cervid TSE being introduced from Norway into Great Britain? Qualitative Risk Assessment September 2016

 

Summary

 

This document is an update of a previous risk assessment carried out in April 2016 to account for the risk of incursion of CWD from the USA and Canada and the assessment of a new pathway, deer urine lures. In April 2016, a report of CWD-like disease was reported in Norway in wild reindeer and later in moose, and this assessment is to review the risk pathways for this new geographic area. Therefore any updates which differ from the assessment of April will appear in red throughout the main text.

 

The Norwegian Veterinary Institute reported a wild reindeer (Rangifer tarandus tarandus) found moribund and which later died in March 2016, had tested positive for the presence of prions. This was the first case of TSE found in a cervid in Europe and the first ever TSE case in a reindeer or caribou. In regions where chronic wasting disease is commonly found in native deer, there have been no reported cases in caribou (related to reindeer), but this is due to the isolation of the populations, rather than the refractivity of the species. The following month, two further cases were reported in wild moose (Alces alces), in a different region and again testing positive for TSE prion protein. In the last month, a fourth case was detected, again in a reindeer in Nordfjell region, this time in a healthy bull, shot for disease surveillance.

 

The new assessment focuses on the potential routes of entry for a cervid TSE (whether confirmed as chronic wasting disease or another related prion) from Norway. The main conclusions from this assessment are:

 

• The likelihood of further cases being found in wild reindeer in Norway is high, if confirmed as CWD as opposed to a spontaneous mutation event.

 

• The likelihood of further cases being found in moose is dependent on whether these are confirmed as a familial case or if related to the TSE in the reindeer. Moose are generally solitary animals so the risk of spread will depend on the level of wider environmental contamination, rather than direct contact with other infected cervids.

 

• The likelihood of spread of a CWD type disease into the farmed reindeer herd or into other farmed cervids in Norway is difficult to assess, and depends on the level of contact between migratory wild reindeer and the semi herded populations in the north or with other farmed cervid species. In the USA, new foci are often detected first in farmed herds, before detection in local wild cervids, so the contact between these two discrete populations is clearly sufficiently high to facilitate transmission in North America. This should be assessed for Norwegian / Scandinavian populations.

 

• The likelihood of spread into other (wild) deer populations in Norway is medium.

 

• The possible routes of spread of TSE from Norway to the UK include movements of live animals, imports of deer-related products (urine lures, meat used as pet food),

 

2 contaminated equipment, including clothing and hunting or skiing equipment and soil surrounding plant imports.

 

• The likelihood of a reindeer imported from Norway to the UK being infected with TSE is very low as they are imported from farmed herds, but there is uncertainty around this level of risk as it is not known if there is disease in the farmed herds.

 

• Other cervid species are not generally imported from Norway to the UK and therefore this is a lower risk pathway. If the pattern of trade changes, or if disease is detected over a wider area, the risk will also change.

 

• For other animals, the movement of pet dogs used for hunting or sledging competitions should be assessed for whether meat of cervid origin is fed to the animals. Where this occurs, this is considered a low risk of disease introduction.

 

• For other pathways, these are a non-negligible risk which is difficult to evaluate without understanding more on the extent of disease in Norway, but is likely to be between very low or low, depending on the pathway. For lures made from natural deer urine, where the provenance of the animal is unknown, the risk is medium for North American origin urine and Norwegian.

 

• As a result of the cases in Norway not all the risk levels have increased in comparison to the same risk pathways with an origin in North America.

 

• Reindeer in the UK are not commonly kept – there are small herds present including in the Cairngorms and Staffordshire and some seasonal imports. However the poor outcome of reindeer kept in captivity in the UK means it is difficult to ascertain whether any may have been infected with prion disease – a fallen stock programme does not exist for such animals at present.

 

• Our previous assessment suggested that of the cervid British species, red deer (Cervus elaphus elaphus) are susceptible to CWD, fallow deer (Dama dama) may be less susceptible and the roe deer (Capreolus capreolus) prion gene codes for susceptibility (and are the most closely related to white-tailed deer). More recent experimental data suggest Sika deer, Chinese Water deer and Muntjac deer may also be susceptible. Therefore, it is likely that given exposure to an infectious dose of CWD or a related prion, deer in GB could become infected.

 

Overall, the probability of importing a TSE into the GB deer herds from Norway and causing infection in British deer is uncertain but likely to be no greater than very low via movement of deer hunters, other tourists and British service personnel; at most, low via live animal imports or imported (non-ruminant) animal feed; very low for the use of lures specifically sourced in Norway and negligible for plant imports. However, if it was imported and (a) deer did become infected with CWD, the consequences would be severe as eradication of the disease is unfeasible, it is clinically indistinguishable from BSE infection in deer and populations of wild and farmed deer would be under threat.

 

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Acknowledgements

 

Thanks to members of the APHA wildlife, TSE and Epidemiology teams, Scottish and Welsh administrations, the Deer Initiative, the Moredun Institute, the British Deer Society and the British Reindeer Herding Society for comments.

 

Background

 

Chronic wasting disease (CWD) is a highly infectious transmissible spongiform encephalopathy (TSE) that is circulating in the wild and farmed cervid populations of North America. It is the only TSE maintained in free-ranging wild animal populations. A feature of CWD is that it is able to transmit both directly (animal-to-animal) and indirectly via the contaminated environment. In particular, CWD prions are able to bind to and survive in the soil in a bio-available form for many years without any decrease in infectivity. This makes eradication of the disease from a wild population very unlikely.

 

Thus far, there have been no reported cases of CWD or other TSE in deer in Great Britain (GB). This is based on surveys of wild and farmed red deer (Cervus elaphus elaphus) carried out several years ago (EFSA, 2011). Given the consequences of CWD observed in North America, it is of high importance that GB remains free of the disease. Further, as the clinical signs of CWD in deer are similar to those of deer experimentally infected with bovine spongiform encephalopathy (BSE), all infected deer would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food chain via affected venison. The public health risk of CWD is not known but current assessments suggest the risk is very low.

 

In 2015, the British Deer Society (BDS) carried out an online survey of BDS and BASC members to gather evidence about the use of deer urine as a lure. Fifteen percent of respondents (~1,800) answered yes about knowing that deer urine was used as a lure. Of the respondents, less than 2% responded yes to using such a product themselves. Of those that use the product, 50% had sourced the product from the USA, while 20% use more than a litre in volume a year and ~70% is natural (as opposed to synthetic).

 

***UPDATE: The report of TSE (CWD like) infection in Norwegian free ranging reindeer and then in European moose has increased the risk to the UK because of the trade in live animals and the different levels of activity for certain pathways, therefore we are reviewing those risk pathways.

 

***In addition, a further pathway was identified by a veterinary colleague overseas, which is that of plant and soil imports as well as for movement of hunting dogs so we have included these in the review.

 

 *** Hazard identification

 

The hazard is identified as TSE in wild European Reindeer and Moose

 

The current geographic range of the cervid TSE, Chronic Wasting Disease (CWD) is the USA and Canada (and occasional outbreaks in South Korea following imports of infected animals).

 

***A new TSE type infection was identified in Norway, in a wild reindeer in March 2016 (NVI, 2016). Until the cases in Norway are confirmed to have a source in USA or Canada, at present we will identify the hazard as a transmissible spongiform encephalopathy (TSE) affecting wild European reindeer and moose.

 

The (found dead) adult reindeer cow showed signs of below-average body condition and detected in connection with capture for GPS-collaring, when it died. It was tested as a routine sample for the national surveillance programme for CWD at the Norwegian Veterinary Institute. Prion disease was confirmed in mid-March by both biochemical and immunohistochemical tests. According to the EURL, most of the samples from different organs were strongly positive for TSE prion protein. Samples were sent to the OIE reference laboratory (Canada) for confirmation as Chronic Wasting Disease. However, based on the widespread distribution of PrPCWD in the brain and the case history, the conclusion has been made that the animal had a spongiform encephalopathy compatible with CWD in the an early clinical stage (Benestad et al., 2016). The animal was found in the Nordfjell region (see map below). In a second event, an adult (pregnant) female moose, (Alces alces) in the Sør-Trøndelag region was found with signs of poor body condition and lack of response to stimuli. It was culled and samples tested by both ELISA and Western blot tests. A third case was detected in another moose, found dead in a river near by a few days later. It also tested positive. There is approximately 300 km distance between the reindeer and the two elk cases. In a fourth case, in August 2016, a reindeer bull was shot and tested positive as part of a surveillance programme, also in the Nordfjell region (Sogn og Fjordane) and a fifth case, again in a reindeer (cow) shot by hunters in the same region was detected in September 2016. The North American moose (also Alces alces) is susceptible to CWD and cases have been found across the moose populations in both the USA and Canada. Alces alces is different to the North American elk (Cervus canadensis) which is also susceptible to CWD.

 

MAP

 

http://wwweng.vetinst.no/eng/Highlights/Detection-of-Chronic-Wasting-Disease-in-two-Norwegian-moose.html

 

http://www.mattilsynet.no/dyr_og_dyrehold/dyrehelse/dyresykdommer/skrantesjuke__cwd_/ny_paavisning_av_skrantesjuke.23759

 

The passive surveillance system in Norway has been running since 2003 and involves testing samples from wild native cervid species of which there are four, red deer (Cervus elaphus), roe deer (Capreolus capreolus), moose (Alces alces) and reindeer (Rangifer tarandus) and from captive deer (Sviland et al, 2015). Red deer predominate along the west coast, wild reindeer live in high mountain areas in southern Norway (see map above). In 2013, the numbers of hunted cervids were nearly 35,000 moose, over 36,000 red deer, over 25,000 roe deer and nearly 8,000 reindeer. There is also a semi-domestic (herded) reindeer population of 250,000 which are located in north Norway and managed by the Sami people, and some of these animals will also be tested. There are 90 deer farms which mainly keep red deer and some keep fallow deer (Dama dama). Scrapie is present in sheep in areas where there are free ranging red deer populations. The number tested each year is very small; in 2014, only 10 deer were tested (all negative), and none of them reindeer. In 2013, again, just ten animals were tested (all negative) (Sviland et al, 2014) and in 2012, 21 animals were tested (all negative) and none were reindeer (Vikoren et al., 2013). This level of surveillance means that when a single positive sample is recorded, it suggests a high prevalence level is likely but the statistical confidence in such sampling is very low. It is not known how many animals have been tested in 2015/2016 for CWD under

 

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the Norwegian programme. The Norwegian Authorities are proposing a large surveillance programme to start in the autumn of 2016, to test around 15,000 animals (moose, roe deer, red deer and reindeer) for fallen stock, hunted animals and at game slaughter houses and approved locations.

 

In Europe and North America, moose or elk (A.alces) are solitary animals, coming together primarily in the mating season, although young stay with their mothers for several months until the next offspring is born. There is a wide level of variation in their movement behaviour with some undertaking very long range migrations, and others being more sedentary. These movements can be categorised as migration, dispersal, nomadism or residence. In Scandinavia, seasonal migration is more likely in northerly populations (regions north of 66oN) than those in the southern regions (regions between 56oN and 66oN) and mean distances decline from ~100 km to 5 km. Seasonal migration can also change with time, depending on the environmental changes, climate or urbanisation. A recent study into the population genetics of Alces alces in Europe suggests there are genetically distinct populations, with the Scandinavian cluster showing low genetic diversity and separate to the other European populations (Niedziałkowska et al. 2016). Nevertheless, the low genetic mixing does not preclude mixing of animals at common grazing areas and therefore having access to contaminated land.

 

The genetic sub-structuring of the A.alces population in Scandinavia could be partly due to geographic barriers, such as the Scandes mountain range which separates Sweden and Norway. This supports the understanding that there is a lower risk of direct disease transmission to other populations of cervids, even of the same species, which are separated by semi-permeable geographic boundaries. However, if there has been widespread environmental contamination over time from a common source of prion, then the risk to other populations will be more difficult to assess.

 

Chronic Wasting Disease was first identified as a clinical disease of captive mule deer in Colorado in 1967 and later classified as a TSE in 1978 (Williams & Miller, 2003). The origin of the disease is unknown and may have been a spontaneous TSE that arose in deer. Currently, natural infections of CWD have been reported in the USA and Canada in mule deer (Odocoileus hemionus hemionus), black-tailed deer (Odocoileus hemionus columbianus), white-tailed deer (Odocoileus virginianus), Rocky Mountain elk (Cervus elphus nelsoni), Shira’s moose (Alces alces shirasi) and mule deer and white-tailed deer hybrids (Hamir et al., 2008).

 

Caribou (Rangifer tarandus caribou, R.t. granti and R.t. goenlandicus) are a subspecies of the Eurasian reindeer, Rangifer tarandus and several populations overlap with the current CWD distribution in Canada. The disease has not been reported in the scientific literature in caribou as natural infections. However, experimental infection of six reindeer resulted in TSE in two of the six animals via oral inoculation (Mitchell et al, 2012). In this study on experimental infection in reindeer, Mitchell and colleagues showed that the two out of three reindeer infected with CWD prion protein (PrP) from brain homogenates of infected white-tailed deer started to show clinical signs between 17 and 18 months after oral inoculation. The same infection route using PrP from infected elk brains did not result in

 

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clinical infection in three further reindeer. Results from histopathology showed PrP present in peripheral lymphoid tissue, in the kidney, the pituitary and adrenal glands, in nerves associated with the gastro-intestinal tract and of course the brain and central nervous system.

 

The widespread distribution in the USA and two Canadian provinces may be detected because of enhanced surveillance but may have increased because of natural movements of cervids and translocation of infected animals by humans (EFSA, 2011). Within affected areas, the prevalence varies. In the endemic area of Wyoming, for example, the prevalence of CWD in mule deer has increased from approximately 11% in 1997 to 36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011). However the separation between caribou populations in Canada and affected cervids is probably the main reason for disease not being detected.

 

The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE). Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.

 

The duration of clinical disease is highly variable and death can occur within 4 weeks but some infected animals may survive as long as a year (Williams, 2005). The incubation period is a minimum of approximately 16 months and is more likely to be between 2 and 4 years (Williams, 2005). In affected American elk, the incubation period is between 1.5 and 3 years after which they become clinically affected and may succumb less than 12 months after initial clinical signs appear (Miller et al., 1998). During the pre-clinical period, the animal is infectious (Almberg et al., 2011).

 

The CWD agent or Prion Protein (PrPCWD) in affected animals is distributed firstly in the gut associated lymphoid tissues, digestive tract (e.g. tonsils, Peyer’s patches, mesenteric lymph nodes) and then in the brain and spinal cord as the disease progresses (Sigurdson, 2008). Prions of CWD have also been found in muscle tissue (Angers et al., 2006) (see Figure 1). The distribution and levels of PrPCWD in tissues differ between species (e.g. American elk versus white tailed or mule deer).

 

Figure 1: Diagram displaying the main organs affected by CWD in infected cervids (http://www.dnr.state.mn.us/mammals/deer/cwd/index.html)

 

Given its propensity to colonise the digestive tract, evidence suggests the prion is excreted in faeces (Safar et al., 2008), urine and saliva potentially leading to direct and indirect transmission between cervid species. Indeed, the disease is transmitted horizontally with high efficiency and circumstantial evidence suggests that environmental contamination with CWD prions contributes to the maintenance of CWD in affected areas (Safar et al., 2008; Nalls et al., 2013). The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008). The efficiency of CWD transmission is unparalleled among TSE diseases (EFSA, 2011). Trifilo et al., (2007), using a murine tg mouse model, established that CWD can be transmitted via the oral route. Indeed, the distribution of PrPres in the orally infected mice (e.g. in the spleen and lymph nodes) mimicked what has been reported in deer developing CWD via natural infection (Trifilo et al., 2007). Modelling studies also support the theory that transmission of CWD in deer herds is maintained by contact with a prion contaminated environment (Almberg et al., 2011). Scavenging of CWD-infected carcasses provides another route of releasing the prion into the environment and exposure of non-cervid species (Sigurdson, 2008). This indirect transmission route is problematic as it not only increases the basic reproductive number but also because there are very few effective mitigation strategies for reducing the risk from indirect transmission. This is due to the fact that the agent is extremely resistant in the environment and able to bind to soil particles making eradication and control of CWD a major obstacle in both farmed and free-ranging cervid populations.

 

The hypothesis that disease can be transmitted between cervid species has been supported by recent experimental studies that have demonstrated that European red deer become infected with CWD after oral inoculation with brain tissue from infected Rocky Mountain elk (Balachandran et al., 2010). Specifically, two of the four 2-month old red deer challenged, showed clinical signs by 585 days p.i. and all deer had CWD prion in the brain, spinal cord and other organs at necropsy (Balachandran et al., 2010). Further, Martin et al., (2009) demonstrated in a similar study of four European red deer, that red deer can

 

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become infected upon inoculation with 5g of infected brain homogenate from four CWD elk and hence the species is susceptible to CWD.

 

Hamir et al., (2008) undertook a study to ascertain if fallow deer (Dama dama), another British deer species, could be experimentally infected with CWD brain suspension from infected elk or white-tailed deer. The authors concluded that it is possible to transmit CWD to fallow deer via the intracerebral route but the pathological features of CWD in the deer differs from those observed in white-tailed deer or elk (Hamir et al., 2008). It was further concluded that it might not be possible to transmit CWD via a more natural route or, alternatively, a higher dose of inoculum is required leading to a longer incubation period (Hamir et al., 2008). However it should be noted that these animals were all sourced from a single breeder therefore genetic diversity would be low and it cannot be ruled out that other fallow deer sourced from other breeders with greater heterogeneity would behave differently.

 

Initial studies into the PRioN Protein (PRNP) gene variability in European red deer and roe deer suggest that these species have a PRNP genetic background that is compatible with TSE susceptibility, including CWD (EFSA, 2011). It is important to note, however, that no experimental studies on roe deer have been conducted verifying this hypothesis.

 

***Recent data on the susceptibility of the other free-ranging deer species present in Britain (muntjac (Muntiacus reevesi), sika (Cervus nippon), Chinese Water deer (Hydropotes inermis)) to CWD also suggests variability in susceptibility for these species (Robinson et al., 2012; Nalls et al., 2013). Further experimental studies would be required to investigate the susceptibility of these species to CWD. Therefore, on the basis of current scientific understanding, it is likely that given exposure to an infectious dose to CWD, most deer species in GB could become infected with CWD.

 

***Sheep and cattle may be exposed to CWD via common grazing areas with affected deer but so far, appear to be poorly susceptible to mule deer CWD (Sigurdson, 2008). In contrast, cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions but no natural CWD infections in cattle have been reported (Sigurdson, 2008; Hamir et al., 2006). It is not known how susceptible humans are to CWD but given that the prion can be present in muscle, it is likely that humans have been exposed to the agent via consumption of venison (Sigurdson, 2008). Initial experimental research suggests that human susceptibility to CWD is low and there may be a robust species barrier for CWD transmission to humans (Sigurdson, 2008), however the risk appetite for a public health threat may still find this level unacceptable. It is apparent, though, that CWD is affecting wild and farmed cervid populations in endemic areas in the USA with some deer populations decreasing as a result.

 

Thus far, CWD is restricted to North America with the exception of imported infected animals into South Korea from Canada. Surveys of wild and farmed cervid populations in the European Union between 2006 and 2010 did not identify any TSEs (EFSA, 2011). As part of this survey, 601 farmed and 598 wild red deer (Cervus elaphus elaphus) were tested (EFSA, 2010). These included clinical/sick animals, fallen stock, healthy

 

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shot/slaughtered animals and road killed animals. Based on the survey results, it was concluded that the prevalence of CWD in the EU is less than 0.5%.

 

Risk Question

 

This risk assessment considers the risk posed to the Great Britain (GB) deer population if chronic wasting disease (CWD) is confirmed in Norway. The specific risk question addressed is:

 

What is the risk of TSE being introduced into Great Britain (GB) from Norway and causing infection in deer?

 

To answer the above question, the risk assessment follows the OIE framework of release (or entry), exposure and consequence assessment. Specifically, it is divided into the three key areas:

 

1. What is the probability of introducing TSE into GB from Norway? [Entry assessment]

 

2. What is the probability of a deer species in GB being exposed to the TSE prion? [Exposure assessment]

 

3. What is the probability of a GB deer species becoming infected with TSE upon exposure to the prion? [Consequence assessment]

 

Risk Assessment

 

Terminology related to the assessed level of risk

 

For the purpose of the risk assessment, the following terminology will apply (OIE, 2004):

 

(PLEASE NOTE THIS CONSIDERS HOW THE RISK IS PERCEIVED BY BSE MRR POLICY, AND THE TRUTHFULNESS TO THOSE COUNTRIES DATA THAT IS/WAS PUT FORTH FOR SAID RISK ASSESSMENT, which in terms of the USA and Canada, is not worth the paper it was written and submitted on. THE OIE HAS NO AUTHORITY TO MAKE ANY COUNTRY SUPPLY TRUTHFUL INFORMATION. imo...TSS)

 

Negligible So rare that it does not merit to be considered

 

Very low Very rare but cannot be excluded

 

Low Rare but does occur

 

Medium Occurs regularly

 

High Occurs often

 

Very high Event occurs almost certainly

 

Entry assessment

 

The routes by which CWD may be introduced into GB from Norway include:

 

• Importation of live deer (including reindeer, other cervids, other animals)

 

• Importation of deer urine lures

 

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• Importation of meat and other products derived from cervid species (e.g. trophy items including antlers, semen)

 

• Importation of animal feed

 

• Hunters and other tourists (skiers and walkers) and British servicemen travelling from affected areas to GB with contaminated equipment (e.g. boots, clothing, knives)

 

• Importation of plants, shrubs and trees with root balls where the soil could be contaminated with prion protein

 

The previous assessments which we carried out concluded the risk of CWD being imported from the USA in meat or other products of animal origin (skins, antlers, semen) was very low and for animal feed as non-negligible (but likely to be very low) because of the requirement for deer entering the food or feed chain to be tested. For deer urine lures, the risk was considered medium. For areas where CWD had only recently been detected there was a very low risk of soil contamination so the risk from movement of equipment of people is considered to be non-negligible but would depend on the frequency of movement. The risk from live animals was not evaluated as live deer imports from the USA and Canada are not allowed under EU Regulation 206/2010. A new pathway is also considered: for plant imports where root balls may be associated with soil contaminated with prion protein.

 

These same controls for meat, products and live animals are not applied to other countries as CWD was geographically restricted. Therefore, this assessment focuses on the following:

 

1) Imports of live animals

 

2) Imports of reindeer meat or products

 

3) Importation of animal feed

 

4) Importation of deer urine lures

 

5) Importation of CWD prion on contaminated equipment and clothing/footwear of hunters or other tourists and British servicemen

 

6) Importation of plants, shrubs and trees

 

Below is a schematic of the different pathways which could potentially lead to entry of CWD to the UK. Note, the human exposure pathway is not included here.

 

For each pathway, the end point is the possible action which could be put in place.

 

SNIP... scheme pathway chart for cwd

 

Trade in live animals

 

Cervids

 

Assuming a maximum incubation period of 2-4 years, the EU Electronic Trade Notification System (TRACES) was interrogated for consignments of reindeer (Rangifer tarandus), moose (Alces alces) and other cervids since 2012. During this period, there have been just two consignments of 28 individual captive reindeer from Norway, as well as 13 consignments of 347 animals from Sweden and 15 consignments of 266 animals from Finland, all from approved holdings. In the case of the Norwegian imports, these are from the north of the country, significantly far from the wild reindeer case and are from semi-domestic herds. There were no recorded reindeer imports from Norway to the UK between 2011 and 2013 or since 2015. In terms of other deer species, there was a single consignment of a zoo species (Pudu) from Sweden, but no other deer species were recorded from the other Nordic or Baltic States. Multiple consignments of deer were traded from other countries in the EU. The importer of the reindeer from Norway has been in contact to assure us his animals are still in a healthy condition.

 

There is no requirement under EEC/92/65 for the animals to be pre-movement tested for CWD or for CWD-herd freedom for movement of any cervid species around the EU or EEA countries, as Europe was believed to be disease free. Nevertheless, the animals must be moved with a veterinary inspection and health certificate and TSE infection of cervidae is listed under Annex A as a notifiable disease of which approved bodies must be free in

 

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order to trade. There is no CWD test approved for live animals, therefore the certifying veterinarian must be confident in signing that the herd is disease free.

 

On the basis of the small number of animals legally imported from Norway and the origin of the reindeer, we consider there is a very low risk of introduction via this route, but this is based on the high level of uncertainty around the possible geographic distribution of disease in Norway and the potential for mixing of wild and captive reindeer and low level of sensitivity in the surveillance programme. Once more surveillance is undertaken to ascertain the level of disease in the wild and semi-domestic herds, this risk level and the uncertainty associated with it can be refined. The level of illegal movement of reindeer or other cervids is not known and cannot be entirely ruled out, if there is deliberate attempt to move the animals without declaring them to the veterinary authorities.

 

Other animals

 

Working dogs may travel to Norway for hunting or sledging competitions. They travel as accompanied animals (under the pet travel scheme) and not with animal health certification (under EEC/92/65). The risk pathway is these dogs being fed raw deer meat and defaecating when they return to the UK. The prion protein would survive the intestine conditions and therefore lead to environmental contamination. It is difficult to assess the likelihood of this pathway, and there are no controls over the use of hunted meat for pet food which is used on farm as opposed to commercially produced. Around 300 dogs are moved under the pet travel scheme every year from Norway to the UK 1, which is less than 1% of all pets moving to the UK. Not all will be dogs involved in hunting or sledging competitions. Therefore we consider this to be a low risk pathway with high level of uncertainty.

 

Other animals which could potentially spread prion proteins to the UK would be from the movements of raptors or other scavenging birds which has fed on a carcase of an infected animal. We consider this risk pathway to be unlikely, given the distance between Norway and the UK.

 

Importation of reindeer meat or products

 

Products of animal origin destined for human consumption are traded from Norway to the EU and UK under the same rules as intra-community trade, under the EEA agreement and are subject to EU Food Law, Regulation (EC) 178/2002. This means there are no health certification requirements or border checks. However, there are still rules under the ABP regulations about not allowing the swill feeding of ruminant material to other ruminants and Food Law covers the general requirements for only safe food to be placed onto the market. Therefore this type of product should not be diverted to animal feed for ruminants. However, poultry and fish feed could contain ruminant material, and this is a pathway

 

1 These numbers are based on figures provided voluntarily to the APHA from pet travel companies and therefore may not be accurate, but are representative.

 

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which may need some further consideration if disease becomes more widely established or detected in farmed cervids.

 

At present, we therefore consider this is no greater than a very low risk pathway, as not only will the product originate in domestic livestock and go through slaughter house checks, but it will be destined for human consumption and not animal feed. In the USA, cervids over 12 months old entering a slaughter house must be tested for CWD, under the USA APHIS Herd Certification Programme, but this is not applied in Norway, therefore there is uncertainty associated with this risk estimate for Norway given the lack of data on the amount of deer or reindeer protein possibly being consigned for human consumption and the lack of surveillance data for the farmed herds in Norway. Food imported for human consumption can be reclassified as category 3 material and be composted or spread as fertiliser, but to understand this pathway better, we consider it would be beneficial to carry out a quantitative risk assessment on the infectious dose and availability of prions through this pathway, should disease be reported more widely.

 

This assessment is only to cover the risk to animal health if the food product were to be diverted to animal feed or mistakenly fed to cervids. It is not the role of this assessment to consider the public health risk associated with meat or other products from cervids infected with CWD. The European Food Safety Authority will be looking into this issue, but the current assessment from the Norwegian Authorities is that it poses a very low risk.

 

Importation of animal feed

 

Animal feed encompasses all feed fed to farmed livestock, horses, pets, farmed fish, zoo and circus animals and also animals living freely in the wild. Currently, legislation for animal feed relating to production, and labelling and composition is harmonised at the EU level and, in GB, is the responsibility of the Food Standards Agency (FSA). In addition, Defra is responsible for ABP Regulations which includes pet food manufacturing regulation.

 

Pet food (i.e. feed for non-ruminants) containing material of animal origin, according to EU Regulation (EC) No. 1069/2009 and its implementing Regulation (EC) 142/2011 on Animal By-Products, must be derived from animals inspected and passed as fit for human consumption prior to slaughter (Category 3 – lowest risk - material). Category 1 material (eg from animals in which the presence of TSE has been confirmed or suspected, or which are derived from animals killed as part of a TSE eradication programme) must not be used for animal feed. Furthermore, the category 3 products are subject to strict microbiological criteria for Enterobacteriaceae and Salmonella. Under the EU Regulation Category 3 processed animal proteins (PAP) must also adhere to a set of standards. More specifically, the pet food must satisfy the following criteria:

 

• The PAP must have been produced in accordance with requirements for placing on the market in the EU.

 

• The PAP must have been sampled and tested to satisfy certain bacteriological criteria in accordance with the Regulations

 

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These requirements apply to canned pet food, processed pet food other than canned pet food, and to dog chews, raw pet food and flavouring innards.

 

*** These regulations do cover entire bodies or parts of wild animals which are carcases and suspected of being diseased, such that they must be disposed of as Category 1 – high risk – material via rendering and incineration and not enter the food or feed chain. It will not cover wild animal carcases or parts from animals not suspected of being diseased or which have been hunted and not collected for disposal. Indeed, as this is considered EU trade there is no requirement for veterinary health certificates or TRACES information as this is part of the European Single Market. Deer antlers are a popular dog chew and deer velvet may contain a low level of prion (Angers et al., 2009) therefore if the antlers are collected from wild deer and imported with the velvet still in place, this could represent a low risk pathway for the pet to consume and defaecate prion proteins.

 

*** CWD-like TSE disease in Norway has only been detected to date in the non-farmed, wild reindeer and moose populations, but there is still uncertainty over whether there could be undetected disease in the farmed herds in the North if they had been in contact with diseased animals in the non-herding areas.

 

*** Overall, therefore, it is considered there is a very low risk that (non-ruminant) animal feed and pet food containing TSE-infected cervid protein is consigned to GB. There is high uncertainty associated with this estimate given the lack of data on the amount of deer or reindeer protein possibly being imported in these products and the lack of surveillance data for the farmed herds in Norway.

 

Importation of deer urine lures

 

*** In our previous assessment, the import of deer urine from the USA as lures from the hunting industry was recognised as a potential route for introduction of disease to the UK / EU (Defra, 2016). It is well established that urine from CWD-infected deer contains CWD infectivity. The urine collected for deer lures has no processing and is immediately refrigerated and bottled (Anon 2015b). There is no inactivation of the CWD agent in the urine and thus all infectivity present at the point of bottling in the USA will still be present at the point of use in the UK. However, under EU rules, (EC Regulation 1069/2009), urine from farmed deer should fall under the definition of “manure” and therefore the import of such a product, if unprocessed, is not allowed, according to Regulation (EC) 142/2011. Nevertheless, the processing required for bottling prior to retail is unlikely to affect the CWD prion and if it did fulfil the requirements in 142/2011, the active ingredient in the urine would no longer be effective. It is therefore presumed that the urine is considered “unprocessed” under EU law. The import and transit of urine from wild deer is not covered by 1069/2009.

 

A survey conducted by the British Deer Society (BDS) in July 2015 suggested small number of hunters in GB were aware of and used deer urine lures (http://www.bds.org.uk/index.php/news-events/135-deer-urine-lure-survey-july-2015 ).

 

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*** It is not known whether Norway is a source of imported deer urine for lures. No information has been found to suggest this. Therefore this route for introduction, while it cannot be discounted, is of no greater risk than for the import of deer lures from countries like the USA where disease is well established in certain deer species.

 

*** Animal urine is not considered a commodity which is subject to animal by-products legislation for imports. Internet sales are common and although a license would be required, there are no conditions for the safe sourcing of such products. Deer urine lures are available in Europe and may be produced from carcases of hunted deer. The use of deer urine produced from a species such as reindeer would be questioned for its value in attracting our native GB deer. This risk is very low, but with a high level of uncertainty, based on the unknown distribution of disease.

 

Movement of hunters, other tourists and British servicemen

 

Probability that the environment in Norway is contaminated with TSE prions

 

At present there are just four animals of two different species and in two different regions which have tested positive for TSE prion proteins in Norway and it is not known what the wider geographic distribution may be or for how long the animals had been infected. However, given the incubation period in experimental infections, the infection was likely to be present for several months, possibly years. Without knowing the original source of disease it is difficult to make an assessment of what the environmental contamination would be. If these were each down to spontaneous mutation events, it may be limited, but it is unlikely for two unrelated species to each have spontaneous events within a few weeks of another. The animals in each case may have been ill for some time, most likely weeks rather than days given the poor condition of the carcases, therefore it is likely the animals were excreting prion protein in various bodily fluids, including urine and faeces which can occur during the pre-clinical phase. This can lead to leaching of prions into the soil and contamination. Animals with clinical signs may show erratic behaviour which can include wandering behaviour, so contamination may be wider than normal host range.

 

CWD (TSE) is excreted in several different bodily fluids and, as demonstrated in experimental studies, can be a source for onward transmission to naïve animals. Infected carcasses decaying naturally in confined areas can also lead to new CWD infections in naïve deer (Sigurdson, 2008). ***There is no reason to expect TSE in reindeer or moose to behave differently, given the experimental infection data and the testing carried out at the EURL and NVI.

 

Once in the environment, TSE prions can bind to soil particles and remain infectious (Saunder et al., 2010). Indeed, Johnson et al., (2006) demonstrated that the disease-associated form of the prion protein can bind to all soil mineral surfaces and is preserved in a bioavailable form. Further, in a later study, Johnson et al., (2007) observed that prions bound to the soil mineral montmorillonite (Mte) significantly enhanced disease penetrance

 

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and reduced the incubation period compared to unbound prions. The reason why binding to Mte or other soil components enhances transmissibility is unclear but it may provide some protection for the prion in the gut against denaturation allowing more agent to be absorbed by the animal (Johnson et al., 2007). Further, binding to the soil particles maintains prions near the soil surface increasing the probability of animal exposure (Russo et al., 2009).

 

In addition to the enhanced infectivity, prions can remain in the soil for several years as the agents are resistant to inactivation by most chemical agents, radiation and heat (Johnson et al., 2006). Seidel et al. (2007), for example, demonstrated that scrapie agent (strain 263K) remains persistent in soil over a period of at least 29 months and remains highly infectious to Syrian hamsters in oral inoculation experiments. In Iceland during an epidemiological investigation of scrapie, a TSE of sheep and goats, Georgsson et al., (2006) reported that the scrapie agent survived on a farm for at least 16 years. However, Russo et al., (2009) demonstrated experimentally that reactive soil components such as manganese oxides may contribute to the inactivation process of TSE prions in soil. The authors did not study CWD prion specifically but the study highlights the complexity of the effect the inorganic and organic constituents in soil may have on prion survival and infectivity.

 

The extent of the contaminated area is unknown at present as it would depend on the range of the infected, clinically ill animals and the presence of undetected infection. However, if there are a larger number of undetected cases of TSE and if animal carcases have not been picked up as fallen stock, but allowed to decompose in situ, or been predated on by other animals, the likelihood of contamination of the land is greater and the risk level increases. Carcases are only one possible source of contamination, as animals may be excreting infection in pre-clinical stages which could be for months or even years. Data from the USA confirm that infection is shed in urine and saliva in particular. The Norwegians are now withdrawing all salt licks because of this (http://www.mattilsynet.no/language/english/animals/additional_legal_measures_to_limit_the_spread_of_chronic_wasting_disease_cwd_in_cervids.23275). Large carnivores, such as lynx, wolves and wolverines may follow herds and prey on sick and weak members of the herd. Foxes, raptors and crows may also contribute to the dissemination.

 

*** In summary, in areas where TSE in cervids has only very recently been reported, it is certain that the soil would be contaminated with CWD prion, but the extent and persistence of that contamination is uncertain but we consider is represents a medium risk in the immediate affected regions and very low to low risk for other areas in Norway.

 

Movement of deer hunters, other outdoor tourists and British service personnel between Norway and GB

 

The probability a person comes into contact with TSE prions varies depending upon their place of residence and/or their involvement with outdoor pursuits (e.g. hunting). In this assessment, focus is given to the following groups of people:

 

• Residents in TSE affected areas travelling to GB (particularly the countryside) and British tourists travelling to TSE affected areas

 

• Hunters travelling between Norway and GB to hunt/stalk deer

 

• British service personnel training in and/or near TSE affected areas

 

All other people (e.g. city tourists and residents) are considered to pose a negligible risk of being exposed to TSE in Norway and, therefore, arriving in GB with contaminated clothing, footwear and/or equipment.

 

The region where the infected reindeer was found is a relatively poorly populated area, but nevertheless is an area where walkers and skiers will visit. Hunting in Norway is a popular sport with many thousand deer shot each year. In order for a foreign national to hunt in GB with your own rifle, a visitor firearms permit has to be obtained from the police force in one of the devolved countries. In 2011, 123 licences were granted by the Scottish Police Force for non-EU residents (BASC, pers. Comm., 2012). This includes not only individuals from North America but also Norway and other non-EU countries (BASC, pers. Comm., 2012). The number of hunters arriving without their own rifle and participating in an organised hunting package/holiday is unknown. The actual number of known hunters either visiting Norway or coming from Norway to the UK is highlighted as a significant data gap. Some of these hunters may also take their own dogs to Norway as highlighted in previous section on live animal imports.

 

As well as tourists, British service personnel frequently move between Norway and GB for winter sports and for alpine training. Consequently, the service personnel have the potential to be in close contact with areas where TSE is present. However data provided by MoD confirm that these affected areas are not used by the British service personnel and therefore there is very low risk via this pathway, with a degree of uncertainty around how widespread is the contamination.

 

*** In summary, given the volume of tourists, hunters and service personnel moving between GB and Norway, the probability of at least one person travelling to/from a TSE affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is considered no greater than low. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is high uncertainty associated with these estimates, mainly around the level of environmental contamination in Norway.

 

Probable amount of CWD prions on contaminated boots and equipment

 

Given that a hunter or tourist walks in areas which are contaminated with TSE, it is possible that they will collect soil on their boots and other equipment. This likelihood will increase if the hunter has shot and handled a TSE infected carcase resulting in contamination of the hunting equipment (e.g. knives) and their clothing and they subsequently arrive in GB with this equipment, footwear and clothing. Furthermore, the soles of hiking boots tend to retain more soil than those of normal shoes. Wilkinson (2010), for example, removed 0.1 g of soil from hiking boots after returning to GB from a 2-month

 

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research visit to Canada. The amount of TSE prion in this amount of soil will depend upon the density of TSE-infected animals excreting prions into the environment and the type of soil; CWD prion binds to clay soil, for example. Animal mortality sites could also be hotspots of TSE prion given the highly infectious nervous system matter entering into the environment and soil (Saunders et al., 2010). Trade in plants, shrubs and trees

 

Live plants (trees, shrubs etc) are moved around the World in considerable quantities and many, including large full-grown trees will have intact root balls which will be covered in soil. As mentioned in the preceding section, prion proteins will be present in soil for many months / years as a result of contamination (Johnson et al., 2007).

 

There are several different categories for imports of stocks of plants, shrubs and trees. According to Eurostat data for codes beginning 0602 since January 2014, there have been only two consignments from Norway of “Live trees and other plants; bulbs, roots and the like; cut flowers and ornamental foliage // Other live plants (including their roots), cuttings and slips; mushroom spawn. // -Other”. Further possible commodities would be “plants for planting” which are generally smaller nursery plants, but we consider these are a very low risk of being in contact with cervids where they are grown, as there would be measures to exclude deer from these high value crops. Further interrogation of the UN COM trade database shows the greatest proportion of imports to the UK of Live trees, plants, bulbs, roots, flowers etc is from Netherlands, and Norway is only a tiny proportion of the total value of our trade. The following figure is a graphical presentation of the imports to the UK of these products. It is also noted that USA and Canada are similarly low exporters to the UK, with less than 50 tons a year combined (ITC, 2016).

 

Prospects for diversification of suppliers for a product imported by United Kingdom in 2015 Product : 06 Live trees, plants, bulbs, roots, cut flowers etc.

 

snip...chart, graph, etc.

 

*** Therefore although this is a potential route for introducing prion proteins into a new area, for this particular situation (Norway to the UK) we consider this is a very low, if not negligible, risk.

 

Exposure assessment

 

Imports of live animals

 

Reindeer management by the Sami in the north of the Scandinavian countries provides only two opportunities a year to handle the reindeer: early summer and autumn. It is these periods which provide many of the non-farmed reindeer for trade. Farmed reindeer can be drawn off and traded at any time, but it is the autumn draw which provides many of the reindeer for export across Europe for the seasonal trade for Christmas markets. In the past they have not thrived particularly in the UK and often succumb to welfare issues when brought in for this seasonal trade. There are a few semi-enclosed herds in the UK – including one in the Cairngorms and one in Staffordshire and there will be plenty of reindeer kept in small numbers in parks and zoos. The exact number is not known as there is no requirement for registration of such animals as there would be for farmed domestic livestock. It is however, estimated that there are around 1,200 – 1,500 reindeer in the UK and there is an intention to start a stud book for the UK animals in the future (British Reindeer Herders Association, pers. comm.). Using a combination of Intra Community Health Certification and performance licencing we have identified several other premises which keep low numbers of reindeer.

 

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Should an infected animal be imported into the resident herds in GB, there is a high probability disease would spread to other in-contact conspecifics, but the level of contact with wild deer may not be as high as for other species (as these are valuable animals). It is nevertheless, difficult to prevent contact between wild and farmed deer and in the USA, disease is often detected first in farmed deer where there are more regular inspections. As there is no official programme of checks on the health status of the reindeer, it is not possible to ascertain whether disease is already present and CWD has not featured as a differential diagnosis for any fallen stock which is reported to APHA, because this was not considered a likely cause of death.

 

Importation of animal feed or products of animal origin

 

Once in GB, the use of animal feed is subject to the TSE Feed Ban and ABP Regulations. The BSE-related feed ban prohibits the feeding of PAP and gelatine from ruminants to ruminants (including farmed deer) or non-ruminant farmed animals. Further, ruminants must not be fed any animal protein or feedstuffs which contain animal protein except for milk, milk-based products and colostrum, eggs and egg products, gelatine from non-ruminants and hydrolysed proteins derived from non-ruminants or from ruminant hides and skins. Therefore, in accordance with the current ban, farmed deer should not be directly exposed to (i.e. fed) imported animal feed containing any PAP.

 

The likelihood of non-ruminant feed or cervid origin POAO being fed accidentally to reindeer or other captive deer in GB is very low.

 

Therefore, assuming this ban is adhered to correctly the risk of farmed deer being exposed to animal feed containing deer protein from Norway is considered negligible but with associated uncertainty.

 

***However, given that non-ruminant feed produced elsewhere (eg the USA) may contain deer and moose PAP, it is theoretically possible that wild deer may be exposed to deer protein in legally imported non-ruminant feed. For this to occur, wild deer would need to access non-ruminant feed (e.g. pig, fish and chicken feed) on farms near their habitat. Alternatively, wild deer may be exposed to TSE prion in the faeces of pets that have consumed and digested imported, contaminated pet feed. Also to be considered is that food may be diverted to composting and the resulting effluate spread on pasture. The frequency in which these routes may occur is unknown and is considered to be a greater than negligible risk with associated uncertainty.***

 

Movement of hunters, other tourists and British servicemen

 

The pathways by which naïve deer can be exposed to CWD contaminated soil and prions on equipment and clothing from people arriving to GB from North America or other affected region are variable and highly uncertain. In principle, in order to expose a deer to CWD prions, the traveller (hunter, tourist or serviceman) would need to transfer the CWD prion from their clothing and/or equipment to the environment in which deer habit. The latter will depend upon the behaviours of returning GB residents or tourists and the probability of entering into and walking around deer territory. In GB, there are two main deer populations (wild and farmed or park deer) each of which will have differing risks of

 

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exposure given the type and frequency of contact with people. Each population type is considered in turn.

 

Consequence assessment

 

Wild deer

 

There are 6 species of wild deer residing in GB including: Red deer (Cervus elaphus), Roe deer (Capreolus capreolus), fallow deer (Dama dama), muntjac (Muntiacus reevesi), sika (Cervus nippon), and Chinese Water deer (Hydropotes inermis). In the UK, enclosed deer herds are principally red deer (Cervus elaphus) on farms and fallow deer (Dama dama) within parks: currently, only one commercial fallow deer farm is known to exist in Great Britain. The number of farmed deer in the UK has grown substantially to about 31,000. The numbers of park deer are unknown but the most recent assessment in 2005 gave an annual cull of around 8,000 deer suggesting about 40,000 park deer. The number of wild deer will be vastly more numerous than the enclosed or captive cervid species, estimated at around 2 million, with an annual cull of over 300,000.

 

Deer hunters, particularly, are most likely to be in direct contact with wild deer and their habitat compared to other tourists and returning GB residents. During the stalking and/or hunting of deer, there is opportunity for TSE prion on the hunter’s boots, clothing and/or equipment to be transferred to the environment. The amount transferred will depend upon the measures taken to remove soil etc from the equipment prior to stalking. Assuming that TSE prion is transferred to the environment, there is an uncertain probability that a deer will come into contact with the TSE prion.

 

Farmed and park deer

 

The Deer Initiative recently carried out an informal review of enclosed deer herds in GB (Deer Initiative, pers comm. Data published in 2015). Current estimates give the number of extant deer parks or collections as 259. The wild roe deer population is the most numerous (see maps in Annex 1), while for farmed deer, fallow deer represent the highest number. Recent estimates provided by the Deer Initiative are:

 

Expected number (by species) held in parks and wild population

 

Species # expected parks Expected population Wild population

 

Sika spp. (C.nippon) 27 2160 ~35,000

 

Red (C. elaphus) 111 12386 >350,000

 

Fallow (D. dama) 196 31849 150-200,000

 

Roe (C.capreolus) - - ~500,000

 

Deer farming is a relatively recent enterprise. There are two systems currently used for managing enclosed deer: park and farm deer systems. In the park system, deer are raised in a park type setting and allowed to roam freely and may be provided with some supplemental feed. Farmed deer, in contrast, following conventional agricultural practices

 

and may be housed in the winter and nutritional supplements are provided where necessary. In this farming system, there are several categories including calf rearers, calf finishers, breeder finishers and producer/processors ( www.bdfpa.org). It is less likely that tourists, deer hunters and British service personnel will come into contact with conventionally farmed deer compared to park deer.

 

*** Reindeer were first introduced in the 1950’s into the Cairngorms, where there is now an established herd. In the 1990’s the imports of live reindeer increased with the popularity of Christmas markets. The imports referred to earlier, from Norway are to one importer where a small herd is maintained for breeding with occasional imported animals from the Northern farmed / semi-domestic herds to improve the bloodlines. Reindeer in this particular herd are fed deer food manufactured in the UK to an adapted Scandinavian recipe. Some feeds are available for other deer that are used and there are a number of manufacturers of pellet feed available in the UK now. Reindeer moss may be imported from a production facility in Norway. This is a dry product which is rehydrated with tap water prior to feeding. This tends to be harvested from close to the Finnish Border away from heavy populations of Reindeer otherwise it wouldn’t be available to pick. A number of people use moss as a treat, training and supplementary feed. Reindeer moss doesn’t store well wet unless frozen so UK imports are dried for longevity. Reindeer in parks in England are generally high fenced and this can reduce contact with local wild deer, particularly nose-to-nose even across single fencing, although the Cairngorm herd is more likely to have some level of interaction with local wildlife. However, it may not be possible to prevent contact with contaminated environment. Reindeer life expectancy is around 10-12 years and can be as long as 18 years in captivity, but is more commonly 14-16 years. There is no requirement for registration of cervidae and their movements, as there is for livestock species.

 

There are therefore several locations in GB where tourists and returning residents may come into contact with park deer.

 

***Nevertheless, there is a relatively low volume of tourists and other travellers moving between Norway and GB, so there are considerably fewer opportunities in comparison to the risk from the USA and Canada for CWD / TSE prions to be transferred from clothing, boots and/or equipment to the environment.

 

It has been observed that multiple exposures to low levels of CWD prions in the environment and increased infectivity of CWD when prions are bound to the soil are influential factors in transmission (Anger et al., 2009). Given the nature of their management, there is a restricted area (or environment) in which park deer inhabit enabling them to have a potentially higher probability of coming into contact with any CWD transferred to the environment by a tourist or returning GB resident compared to wild deer in a free-ranging environment.

 

***Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.

 

***Nevertheless, the risk is not increased by this case in Norway, as to that posed by visitors from the USA and Canada.

 

Exposure of UK deer to soil, feed or infected urine

 

Whether the amount of CWD prion that could be transferred from clothing, boots and/or other equipment into the deer’s environment is enough to induce infection given that the infectious dose is extremely small (Saunders et al., 2010) is uncertain. However, given that the amount of soil ingested is likely to be very small, the probability of ingesting an infectious dose via this route is considered no greater than very low. The probability of ingesting an infectious dose via consumption of non-ruminant feed is likely to be higher and may be very low, with associated uncertainty.

 

Although our previous risk assessment considered the risk of importing TSE through imports of deer urine for lures as medium entry assessment, and the likely exposure assessment as very low, the risk is not increased as a result of this case in Norway. The import of unprocessed deer urine is still an illegal product and should not be imported into the EU, no matter what the source of the animals.

 

The CWD agent is relatively dilute in deer urine compared to brain and spinal cord material with 1 ic LD50 per 10 ml. The LD50 determined by Henderson et al. (2015) is an intracerebral ID50 in cervidized transgenic mice. The oral ID50 in deer would be in a much larger volume of urine, because the oral route may be 100,000-fold less efficient than the intracerebral route in terms of TSE transmission (Gale et al 1998). Thus, in terms of oral LD50, there may be one in a 1,000,000 ml i.e. 1 m3 or 1,000 litre volume of deer urine. Therefore a deer would have to ingest 1,000 litres of urine to have a 50% chance of being infected through the oral route. Of course the CWD agent would be concentrated as the urine evaporated from the tree trunk.

 

The main sources of uncertainty are:-

 

1. The amount of urine ingested by the deer. A bottle of Tink’s “69 Doe-in-Rut Buck” is about 30 ml and boasts 100% natural doe oestrous urine (Anon 2105d). A bottle from an infected doe would thus contain about 3 i.c. LD50 units. The bottles come with an easy to use squirt top, so it seems relatively small amounts are used. There are also gel forms which do not freeze as fast, last longer in the rain, and do not dry out so quickly.

 

2. The magnitude of oral/i.c. barrier

 

Assuming a deer drank 10 ml of urine from each 30 ml bottle, then that deer would ingest 10-5 LD5. It is generally assumed that there is no threshold dose for TSEs (Gale 2006) and the risk of CWD infection in GB deer per 30 ml bottle imported is therefore 0.69 x 10-5 (Gale 1998). This is very low.

 

Risk of infection of deer in GB per 30 ml bottle of deer urine lure imported from the USA

 

Step in pathway Risk Uncertainty

 

Entry (probability a 30-ml bottle contains urine from an infected deer) Medium Low

 

Exposure (probability some of 30-ml bottle is ingested by UK deer) Medium High

 

Consequence (probability that deer is infected given exposure) Very low Medium

 

Overall risk Very low High

 

*** Overall the risk of a deer in GB being infected per 30 ml bottle of urine imported from the USA is very low, albeit with high uncertainty.

 

*** The next question is how much deer urine is imported into GB from the USA per year. If there are n 30-ml bottles, then the risk is calculated as:-

 

pn_bottle = 1 - (1 – pone_bottle)n

 

***Hundreds of gallons of urine are sold every year just by one company in the USA (Anon 2015b). It is difficult, however, to estimate the volume of deer urine imported into the UK per year from the USA. That some respondents to the BDS survey reported they used upwards of 1litre per year, is consistent with importation of a high number of 30-ml bottles. Assuming that the number, n, of 30-ml bottles imported annually is high, then the risk of CWD infection in at least one deer in the UK per year will be medium (given the probability, of infection of a UK deer from one 30-ml bottle is very low (see Table 1)).

 

***Overall it is concluded that the annual risk of at least one infection of deer in the UK with CWD from deer urine lures imported from the USA is medium. This assumes a high number of 30 ml bottles imported per year from all areas of the USA.

 

***The case in Norway does not increase this risk at present. A quick internet search continues to suggest the main source of deer urine lures is the USA, nevertheless, in the BDS survey, half of respondents sourced deer urine from North America and a quarter from Europe.

 

 Control and risk management options

 

The highest level of uncertainty is around the source and level of disease in Norway and whether it is limited to the wild reindeer herds or if it has spread into either other species of wild deer or the herder reindeer in the North. As there is so little by way of surveillance in the EU and surrounding countries, it is not possible to say whether this is a wider problem.

 

If these were spontaneous mutations, a form of “atypical CWD”, then these may be rare and isolated cases. However the animals concerned were in two regions, of two species and not particularly old, so it may be more likely the source is an imported prion infection. If this has an origin in the USA or Canada, it is important to try to understand the source and therefore whether there are likely to be more cases over a wider area, where the import event may also have taken place.

 

If it were to be found in the farmed or herded cervid populations, it will be necessary to put in place some import controls along the lines of those in place for imports of live animals and cervid meat from the USA and Canada. As it is, Norway voluntarily stopped signing trade certificates for all cervid species and authorisation is required to move cervids

 

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between counties within Norway

 

(http://www.mattilsynet.no/language/english/animals/additional_legal_measures_to_limit_the_spread_of_chronic_wasting_disease_cwd_in_cervids.23275).

 

If the geographic distribution is wider and the environment heavily contaminated, to reduce the potential amount of TSE prion entering GB on boots and clothing, it is important to meticulously clean off all adherent material prior to departing from an affected area and where possible, equipment should be soaked in a solution of bleach that has 20,000 parts per million of active chlorine for one hour. However, it is acknowledged it is impractical to soak hunting boots, clothing or firearms, for example, in strong concentrations of bleach.

 

For deer urine lures, the import of unprocessed natural deer urine is not allowed under the EU Animal By-Product legislation. However the import of such commodities including internet sales would still require licenses, but it would be a voluntary action on behalf of the importer to ensure the urine is sourced from safe herds. One option is to ensure the sourcing is from herds in the USA which are currently registered under the National CWD Herd Certification Programme which has recently been introduced in the USA. Otherwise the use of synthetic or domestic-produced lures should be promoted by the industry and stakeholder groups.

 

Areas identified which are not fully understood are those of pets eating meat from infected animals and of food diverted to Category 3 for composting. Both would benefit from further investigation if the results of the Norwegian surveillance suggest wider distribution.

 

Diagnosis of CWD / TSE in cervids

 

Conventional immunohistochemistry (IHC) testing for TSE’s involves using cadavers to remove the brainstem as the most sensitive organ to show the distribution of prion protein in the nervous and associated tissue is typical of the pathology associated with the species-specific TSE. The ELISA for CWD has similar sensitivity and specificity as the IHC test. Both tests use a protease step which can reduce sensitivity. This means that surveillance cannot be carried out on live animals. There is a considerable need for a fast, reliable antemortem test.

 

There is no current fallen stock scheme for testing deer species in the UK and there is no European Reference Lab for CWD (although the EURL for TSE’s is APHA, Weybridge).

 

Conclusions

 

There is significant uncertainty associated with estimating the risk of TSE entering the UK from Norway via imports of live animals, movement of people (tourists, hunters and British servicemen) and importation of animal feed or urine lures. This stems from the lack of data on the current distribution of disease, not only in wild reindeer and moose in Norway, but also in herded animals and more widely in other wild cervids across the region and

 

27

 

neighbouring counties. Notwithstanding this uncertainty, the probability of importing TSE into GB from Norway and causing infection in British deer is likely to be greater than negligible via movement of deer hunters, other tourists and British servicemen and low via live animals, very low via products of animal origin or imported (non-ruminant) animal feed. However the risk of natural deer urine lures from Norway is lower than for those products sourced from the USA, where the risk of containing CWD PrP is considered medium (reflected by some US States banning the use) and the probability of such a commodity, if used in significant volumes, leading to CWD infection in GB populations is considered to be very low (potentially reduced susceptibility in certain species and limited use by hunters and stalkers in GB) but with a high level of uncertainty. Nevertheless, the voluntary restrictions on sourcing urine lures from USA and Canada should be extended to Norwegian sources, as a precautionary measure.

 

The consequences of CWD, however, are severe with the minimal possibility of eradicating the disease from a wild cervid population and populations of both wild and farmed deer in the UK would be under threat.

 

Current research indicates that of the six free-ranging deer species in the UK, red deer, and muntjac are susceptible to CWD, while roe deer, which is the closest related to white-tailed deer, Japanese sika and Chinese water deer are likely to also be susceptible. Farmed fallow deer are numerous in the UK and while those studied to date have lacked the PRNP polymorphisms associated with higher susceptibility to CWD, our populations are genetically heterogenous so the risk of infection cannot be ruled out. Wild roe deer are even more numerous, so again, understanding the susceptibility of this species will be important. The new case in reindeer in Norway taken alongside existing experimental data suggests that this species is also at risk.

 

It is important, therefore, that the risk of these species being exposed to TSE is minimised by taking appropriate precautionary measures.

 

Change in risk level for entry assessment (and level of uncertainty) from previous risk assessment (Defra, 2016)

 

 Change in risk level for entry assessment (and level of uncertainty) from previous risk assessment (Defra, 2016)

 

Pathway USA and Canada Norway

 

Live animals - cervids Not assessed as not possible Very low (high)

 

Live animal - other Not assessed as new pathway Low (high)

 

Products of Animal Origin Very low (low) Very low (medium)

 

Animal Feed Greater than negligible (medium) Very low (high)

 

Urine lures Medium (low) Medium (medium)

 

Equipment and people Greater than negligible (medium) Greater than negligible (medium)

 

Plants Not assessed as new pathway Negligible (low)

 

References

 

snip...see full text here;

 

https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/557205/qra-tse-reindeer-norway.pdf

 

March 2016

 

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 

https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/514401/qra-chronic-wasting-disease.pdf

 

Summary

 

The previous assessment concentrated on the incursion of disease from North America through the imports of animal feed or the movement of contaminated clothing, footwear and equipment. The results suggested that import of pet feed was a non-negligible risk, but given the unlikely contact of resident deer in GB with such non-ruminant feed, this was considered overall a negligible to very low risk. The movement of contaminated clothing, footwear or equipment (particularly hunting equipment) could pose a very low risk, although the volume of contaminated soil which would need to be ingested to give rise to an infection is likely to be higher than would be present. There is a variable level uncertainty in all these assessments.

 

The new assessment focuses on an additional potential route of entry: the importation of natural deer urine lures. The main conclusions from this assessment are:

 

***In areas of North America where CWD has been reported, given that CWD is excreted in faeces, saliva, urine and blood, and survives in the environment for several years there is a medium probability that the deer urine in North America contains CWD (high uncertainty; depends on the source of deer used for production).

 

***The risk of a deer in GB being infected per 30 ml bottle of urine imported from the USA is very low, albeit with high uncertainty. Overall it is concluded that the risk of at least one infection of deer in the UK with CWD per year from deer urine lures imported from the USA is medium. This assumes a high number of 30 ml bottles imported per year from all areas of the USA.

 

***None of the species affected by CWD in North America are present in GB. For a British species to become infected with CWD following exposure, the dose and inherent susceptibility of the species will be important. Based on current scientific evidence Red deer (Cervus elaphus elaphus) are susceptible to CWD, Fallow deer (Dama dama) are likely to be less susceptible and Roe deer (Capreolus capreolus) have a gene conferring susceptibility. Therefore, it is likely that given exposure to an infectious dose of CWD, deer in GB could become infected with CWD.

 

Overall, the probability of importing CWD into GB from North America and causing infection in British deer is uncertain but likely to be negligible to very low via movement of deer hunters, other tourists and British servicemen and very low via imported (non-

 

2

 

ruminant) animal feed and medium for the use of lures. However, if it was imported and (a) deer did become infected with CWD, the consequences would be severe as eradication of the disease is impossible, it is clinically indistinguishable from BSE infection in deer (Dalgleish et al., 2008) and populations of wild and farmed deer would be under threat.

 

The USA has implemented a Herd Certification Programme for farmed and captive cervids. So far, 29 States are approved for HCP status (APHIS, 2015). The list includes States such as Colorado, where CWD is present, therefore it is recommended that any sourcing of such natural urine lures should be not only from States with an HCP programme, but also from a herd which is registered as being regularly tested free of CWD.

 

Animal urine is not considered a commodity which is subject to animal by-products legislation for imports. Internet sales are common and although a license would be required, there are no conditions for the safe sourcing of such products. Deer urine lures are also available in Europe and may be produced from carcases of hunted deer. The use of deer urine produced from a species not present in Europe (such as white tailed deer) is questioned for its value with native GB deer according to the British Deer Society survey.

 

Background

 

https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/514401/qra-chronic-wasting-disease.pdf

 

April 07, 2016

 

Thursday, April 07, 2016

 

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 

http://chronic-wasting-disease.blogspot.com/2016/04/what-is-risk-of-chronic-wasting-disease.html

 

Friday, December 14, 2012

 

DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012

 

snip...

 

In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

 

Animals considered at high risk for CWD include:

 

1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and

 

2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.

 

Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.

 

The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.

 

Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.

 

There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.

 

snip...

 

36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).

 

The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).

 

Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.

 

snip...

 

The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).

 

snip...

 

In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.

 

snip...

 

In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.

 

snip...

 

Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.

 

snip...

 

What is the risk of chronic wasting disease being introduced into Great Britain? A Qualitative Risk Assessment October 2012

 

http://webarchive.nationalarchives.gov.uk/20130822084033/http://www.defra.gov.uk/animal-diseases/files/qra_chronic-wasting-disease-121029.pdf

 

2016

 

*** Title: Pathological features of chronic wasting disease in reindeer and demonstration of horizontal transmission ***

 

Author

 

item Moore, Sarah item Kunkle, Robert item West greenlee, Mary item Nicholson, Eric item Richt, Juergen item Hamir, Amirali item Waters, Wade item Greenlee, Justin

 

Submitted to: Emerging Infectious Diseases Publication Type: Peer reviewed journal Publication Acceptance Date: 8/29/2016 Publication Date: N/A Citation:

 

Interpretive Summary: Chronic wasting disease (CWD) is a fatal neurodegenerative disease that occurs in farmed and wild cervids (deer and elk) of North America and was recently diagnosed in a single free-ranging reindeer (Rangifer tarandus tarandus) in Norway. CWD is a transmissible spongiform encephalopathy (TSE) that is caused by infectious proteins called prions that are resistant to various methods of decontamination and environmental degradation. Little is known about the susceptibility of or potential for transmission amongst reindeer. In this experiment, we tested the susceptibility of reindeer to CWD from various sources (elk, mule deer, or white-tailed deer) after intracranial inoculation and tested the potential for infected reindeer to transmit to non-inoculated animals by co-housing or housing in adjacent pens. Reindeer were susceptible to CWD from elk, mule deer, or white-tailed deer sources after experimental inoculation. Most importantly, non-inoculated reindeer that were co-housed with infected reindeer or housed in pens adjacent to infected reindeer but without the potential for nose-to-nose contact also developed evidence of CWD infection. This is a major new finding that may have a great impact on the recently diagnosed case of CWD in the only remaining free-ranging reindeer population in Europe as our findings imply that horizontal transmission to other reindeer within that herd has already occurred. Further, this information will help regulatory and wildlife officials developing plans to reduce or eliminate CWD and cervid farmers that want to ensure that their herd remains CWD-free, but were previously unsure of the potential for reindeer to transmit CWD.

 

Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal prion disease of cervids. Reindeer (Rangifer tarandus tarandus) are susceptible to CWD following oral challenge, and CWD was recently reported in a free-ranging reindeer of Norway. Potential contact between CWD-affected cervids and Rangifer species that are free-ranging or co-housed on farms presents a potential risk of CWD transmission. The aims of this study were to 1) investigate the transmission of CWD from white-tailed deer (Odocoileus virginianus; CWDwtd), mule deer (Odocoileus hemionus; CWDmd), or elk (Cervus elaphus nelsoni; CWDelk) to reindeer via the intracranial route, and 2) to assess for direct and indirect horizontal transmission to non-inoculated sentinels. Three groups of 5 reindeer fawns were challenged intracranially with CWDwtd, CWDmd, or CWDelk. Two years after challenge of inoculated reindeer, non-inoculated negative control reindeer were introduced into the same pen as the CWDwtd inoculated reindeer (direct contact; n=4) or into a pen adjacent to the CWDmd inoculated reindeer (indirect contact; n=2). Experimentally inoculated reindeer were allowed to develop clinical disease. At death/euthanasia a complete necropsy examination was performed, including immunohistochemical testing of tissues for disease-associated CWD prion protein (PrPcwd). Intracranially challenged reindeer developed clinical disease from 21 months post-inoculation (months PI). PrPcwd was detected in 5 out of 6 sentinel reindeer although only 2 out of 6 developed clinical disease during the study period (< 57 months PI). We have shown that reindeer are susceptible to CWD from various cervid sources and can transmit CWD to naïve reindeer both directly and indirectly.

 

https://www.ars.usda.gov/research/publications/publication/?seqNo115=328261

 

Monday, September 05, 2016

 

Pathological features of chronic wasting disease in reindeer and demonstration of horizontal transmission Major Findings for Norway

 

http://chronic-wasting-disease.blogspot.com/2016/09/pathological-features-of-chronic.html

 

Wednesday, September 28, 2016

 

*** Norway sides with OIE, decides to expose millions of consumers to the ATYPICAL BSE SRM TSE Prion aka mad cow type disease ***

 

http://bse-atypical.blogspot.com/2016/09/norway-sides-with-oie-decides-to-expose.html

 

Thursday, September 22, 2016

 

NORWAY DETECTS 5TH CASE OF CHRONIC WASTING DISEASE CWD TSE PRION Skrantesjuke

 

http://chronic-wasting-disease.blogspot.com/2016/09/norway-detects-5th-case-of-chronic.html

 

Saturday, September 03, 2016

 

NORWAY Regulation concerning temporary measures to reduce the spread of Chronic Wasting Disease (CWD) as 4th case of skrantesjuke confirmed in Sogn og Fjordane

 

http://chronic-wasting-disease.blogspot.com/2016/09/norway-regulation-concerning-temporary.html

 

Wednesday, August 31, 2016

 

*** NORWAY CONFIRMS 4TH CASE OF CHRONIC WASTING DISEASE CWD TSE PRION IN SECOND CARIBOU

 

http://chronic-wasting-disease.blogspot.com/2016/08/norway-confirms-4th-case-of-chronic.html

 

Wednesday, August 31, 2016

 

NORWAY CONFIRMS 4TH CASE OF CHRONIC WASTING DISEASE CWD TSE PRION IN SECOND CARIBOU

 

http://chronic-wasting-disease.blogspot.com/2016/08/norway-confirms-4th-case-of-chronic.html

 

Tuesday, August 02, 2016

 

Chronic wasting disease of deer – is the battle to keep Europe free already lost?

 

http://chronic-wasting-disease.blogspot.com/2016/08/chronic-wasting-disease-of-deer-is.html

 

Tuesday, June 14, 2016

 

*** Chronic Wasting Disease (CWD) in a moose from Selbu in Sør-Trøndelag Norway ***

 

http://chronic-wasting-disease.blogspot.com/2016/06/chronic-wasting-disease-cwd-in-moose.html

 

Thursday, July 07, 2016

 

Norway reports a third case Chronic Wasting Disease CWD TSE Prion in 2nd Norwegian moose

 

14/06/2016 - Norway reports a third case

 

http://chronic-wasting-disease.blogspot.com/2016/07/norway-reports-third-case-chronic.html

 

Saturday, July 16, 2016

 

Chronic wasting Disease in Deer (CWD or Spongiform Encephalopathy) The British Deer Society 07/04/2016

 

Red Deer Ataxia or Chronic Wasting Disease CWD TSE PRION?

 

could this have been cwd in the UK back in 1970’S ???

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1982/07/05001001.pdf

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1982/07/28001001.pdf

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1983/03/01001001.pdf

 

http://www.tandfonline.com/doi/pdf/10.1080/00480169.1979.34665

 

SEE FULL TEXT ;

 

http://chronic-wasting-disease.blogspot.com/2016/07/chronic-wasting-disease-in-deer-cwd-or.html

 

Tuesday, April 12, 2016

 

The first detection of Chronic Wasting Disease (CWD) in Europe free-ranging reindeer from the Nordfjella population in South-Norway.

 

http://chronic-wasting-disease.blogspot.com/2016/04/the-first-detection-of-chronic-wasting.html

 

Saturday, April 9, 2016

 

The Norwegian Veterinary Institute (NVI, 2016) has reported a case of prion disease Cervid Spongiform Encephalopathy detected in free ranging wild reindeer (Rangifer tarandus tarandus)

 

Department for Environment, Food and Rural Affairs

 

http://transmissiblespongiformencephalopathy.blogspot.com/2016/04/the-norwegian-veterinary-institute-nvi.html

 

Wednesday, September 7, 2016

 

*** An assessment of the long-term persistence of prion infectivity in aquatic environments

 

http://transmissiblespongiformencephalopathy.blogspot.com/2016/09/an-assessment-of-long-term-persistence.html

 

Friday, September 02, 2016

 

*** Chronic Wasting Disease Drives Population Decline of White-Tailed Deer

 

http://chronic-wasting-disease.blogspot.com/2016/09/chronic-wasting-disease-drives.html

 

*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years ***

 

Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3

 

http://jgv.sgmjournals.org/content/87/12/3737.full

 

Using in vitro prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.

 

Claudio Soto

 

Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.

 

Prion and prion-like proteins are misfolded protein aggregates with the ability to selfpropagate to spread disease between cells, organs and in some cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are mostly composed by a misfolded form of the prion protein (PrPSc), which propagates by transmitting its misfolding to the normal prion protein (PrPC). The availability of a procedure to replicate prions in the laboratory may be important to study the mechanism of prion and prion-like spreading and to develop high sensitive detection of small quantities of misfolded proteins in biological fluids, tissues and environmental samples. Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion replication in the test tube. PMCA is a platform technology that may enable amplification of any prion-like misfolded protein aggregating through a seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of one single molecule of infectious PrPSc and propagate prions that maintain high infectivity, strain properties and species specificity. Using PMCA we have been able to detect PrPSc in blood and urine of experimentally infected animals and humans affected by vCJD with high sensitivity and specificity. Recently, we have expanded the principles of PMCA to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to study the utility of this technology to detect Aβ and α-syn aggregates in samples of CSF and blood from patients affected by these diseases.

 

=========================

 

***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.

 

========================

 

Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.

 

https://prion2015.files.wordpress.com/2015/05/programguide1.pdf

 

see ;

 

with CWD TSE Prions, I am not sure there is any absolute yet, other than what we know with transmission studies, and we know tse prion kill, and tse prion are bad. science shows to date, that indeed soil, dirt, some better than others, can act as a carrier. same with objects, farm furniture. take it with how ever many grains of salt you wish, or not. if load factor plays a role in the end formula, then everything should be on the table, in my opinion. see ;

 

***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.

 

Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.

 

https://prion2015.files.wordpress.com/2015/05/programguide1.pdf

 

see ;

 

http://www.tandfonline.com/doi/pdf/10.4161/pri.28467

 

Oral Transmissibility of Prion Disease Is Enhanced by Binding to Soil Particles

 

Author Summary

 

Transmissible spongiform encephalopathies (TSEs) are a group of incurable neurological diseases likely caused by a misfolded form of the prion protein. TSEs include scrapie in sheep, bovine spongiform encephalopathy (‘‘mad cow’’ disease) in cattle, chronic wasting disease in deer and elk, and Creutzfeldt-Jakob disease in humans. Scrapie and chronic wasting disease are unique among TSEs because they can be transmitted between animals, and the disease agents appear to persist in environments previously inhabited by infected animals. Soil has been hypothesized to act as a reservoir of infectivity and to bind the infectious agent. In the current study, we orally dosed experimental animals with a common clay mineral, montmorillonite, or whole soils laden with infectious prions, and compared the transmissibility to unbound agent. We found that prions bound to montmorillonite and whole soils remained orally infectious, and, in most cases, increased the oral transmission of disease compared to the unbound agent. The results presented in this study suggest that soil may contribute to environmental spread of TSEs by increasing the transmissibility of small amounts of infectious agent in the environment.

 

https://www.aphis.usda.gov/emergency_response/downloads/tools/johnson%20et%20al%20prions%20in%20soil.pdf

 

tse prion soil

 

http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0058630

 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3567181/pdf/ppat.1003113.pdf

 

http://www.nature.com/srep/2015/150210/srep08358/full/srep08358.html?WT.ec_id=SREP-639-20150217

 

http://www.cell.com/cell-reports/pdfExtended/S2211-1247(15)00437-4

 

Wednesday, December 16, 2015

 

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

 

http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full

 

The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.

 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4397813/pdf/13567_2015_Article_176.pdf

 

>>>Particle-associated PrPTSE molecules may migrate from locations of deposition via transport processes affecting soil particles, including entrainment in and movement with air and overland flow. <<<

 

Fate of Prions in Soil: A Review

 

Christen B. Smith, Clarissa J. Booth, and Joel A. Pedersen*

 

Several reports have shown that prions can persist in soil for several years. Significant interest remains in developing methods that could be applied to degrade PrPTSE in naturally contaminated soils. Preliminary research suggests that serine proteases and the microbial consortia in stimulated soils and compost may partially degrade PrPTSE. Transition metal oxides in soil (viz. manganese oxide) may also mediate prion inactivation. Overall, the effect of prion attachment to soil particles on its persistence in the environment is not well understood, and additional study is needed to determine its implications on the environmental transmission of scrapie and CWD.

 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3160281/

 

P.161: Prion soil binding may explain efficient horizontal CWD transmission

 

Conclusion. Silty clay loam exhibits highly efficient prion binding, inferring a durable environmental reservoir, and an efficient mechanism for indirect horizontal CWD transmission.

 

https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf

 

>>>Another alternative would be an absolute prohibition on the movement of deer within the state for any purpose. While this alternative would significantly reduce the potential spread of CWD, it would also have the simultaneous effect of preventing landowners and land managers from implementing popular management strategies involving the movement of deer, and would deprive deer breeders of the ability to engage in the business of buying and selling breeder deer. Therefore, this alternative was rejected because the department determined that it placed an avoidable burden on the regulated community.<<<

 

Wednesday, December 16, 2015

 

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

 

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

 

Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4, Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1

 

1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge, Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK

 

Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial protein misfolding cyclic amplification (sPMCA) have suggested that objects on a scrapie affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing, and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation, sheep became infected when exposed to either a water trough (four out of five) or to objects used for rubbing (four out of seven). This field furniture had been used by the scrapie-infected flock 8 weeks earlier and had previously been shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 23) through exposure to contaminated field furniture placed within pasture not used by scrapie-infected sheep for 40 months, even though swabs from this furniture tested negative by PMCA. This infection rate decreased (1 out of 12) on the same paddock after replacement with clean field furniture. Twelve grazing sheep exposed to field furniture not in contact with scrapie-infected sheep for 18 months remained scrapie free. The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.

 

snip...

 

Discussion

 

Classical scrapie is an environmentally transmissible disease because it has been reported in naïve, supposedly previously unexposed sheep placed in pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the vector for disease transmission is not known, soil is likely to be an important reservoir for prions (2) where – based on studies in rodents – prions can adhere to minerals as a biologically active form (21) and remain infectious for more than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in mule deer housed in paddocks used by infected deer 2 years earlier, which was assumed to be through foraging and soil consumption (23).

 

Our study suggested that the risk of acquiring scrapie infection was greater through exposure to contaminated wooden, plastic, and metal surfaces via water or food troughs, fencing, and hurdles than through grazing. Drinking from a water trough used by the scrapie flock was sufficient to cause infection in sheep in a clean building. Exposure to fences and other objects used for rubbing also led to infection, which supported the hypothesis that skin may be a vector for disease transmission (9). The risk of these objects to cause infection was further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of the paddocks, which contained metal hurdles, a metal lamb creep and a water trough in contact with the scrapie flock up to 8 weeks earlier, whereas no infection had been demonstrated previously in sheep grazing on this paddock, when equipped with new fencing and field furniture. When the contaminated furniture and fencing were removed, the infection rate dropped significantly to 8% of 12 sheep, with soil of the paddock as the most likely source of infection caused by shedding of prions from the scrapie-infected sheep in this paddock up to a week earlier.

 

This study also indicated that the level of contamination of field furniture sufficient to cause infection was dependent on two factors: stage of incubation period and time of last use by scrapie-infected sheep. Drinking from a water trough that had been used by scrapie sheep in the predominantly pre-clinical phase did not appear to cause infection, whereas infection was shown in sheep drinking from the water trough used by scrapie sheep in the later stage of the disease. It is possible that contamination occurred through shedding of prions in saliva, which may have contaminated the surface of the water trough and subsequently the water when it was refilled. Contamination appeared to be sufficient to cause infection only if the trough was in contact with sheep that included clinical cases. Indeed, there is an increased risk of bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA. Although ultraviolet light and heat under natural conditions do not inactivate prions (26), furniture in contact with the scrapie flock, which was assumed to be sufficiently contaminated to cause infection, did not act as vector for disease if not used for 18 months, which suggest that the weathering process alone was sufficient to inactivate prions.

 

PrPSc detection by sPMCA is increasingly used as a surrogate for infectivity measurements by bioassay in sheep or mice. In this reported study, however, the levels of PrPSc present in the environment were below the limit of detection of the sPMCA method, yet were still sufficient to cause infection of in-contact animals. In the present study, the outdoor objects were removed from the infected flock 8 weeks prior to sampling and were positive by sPMCA at very low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on any of the objects above the background of the assay. False positive reactions with sPMCA at a low frequency associated with de novo formation of infectious prions have been reported (27, 28). This is in contrast to our previous study where we demonstrated that outdoor objects that had been in contact with the scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly more positive by the assay compared to analogous samples from the scrapie-free farm. This discrepancy could be due to the use of a different sPMCA substrate between the studies that may alter the efficiency of amplification of the environmental PrPSc. In addition, the present study had a longer timeframe between the objects being in contact with the infected flock and sampling, which may affect the levels of extractable PrPSc. Alternatively, there may be potentially patchy contamination of this furniture with PrPSc, which may have been missed by swabbing. The failure of sPMCA to detect CWD-associated PrP in saliva from clinically affected deer despite confirmation of infectivity in saliva-inoculated transgenic mice was associated with as yet unidentified inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is affected by other substances in the tested material. In addition, sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from furniture exposed to weather, which is supported by the observation that PrPSc was detected by sPMCA more frequently in indoor than outdoor furniture (12). A recent experimental study has demonstrated that repeated cycles of drying and wetting of prion-contaminated soil, equivalent to what is expected under natural weathering conditions, could reduce PMCA amplification efficiency and extend the incubation period in hamsters inoculated with soil samples (30). This seems to apply also to this study even though the reduction in infectivity was more dramatic in the sPMCA assays than in the sheep model. Sheep were not kept until clinical end-point, which would have enabled us to compare incubation periods, but the lack of infection in sheep exposed to furniture that had not been in contact with scrapie sheep for a longer time period supports the hypothesis that prion degradation and subsequent loss of infectivity occurs even under natural conditions.

 

In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.

 

Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification

 

http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full

 

Wednesday, December 16, 2015

 

*** Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission ***

 

http://scrapie-usa.blogspot.com/2015/12/objects-in-contact-with-classical.html

 

*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years ***

 

Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3

 

http://jgv.sgmjournals.org/content/87/12/3737.full

 

>>>Another alternative would be an absolute prohibition on the movement of deer within the state for any purpose. While this alternative would significantly reduce the potential spread of CWD, it would also have the simultaneous effect of preventing landowners and land managers from implementing popular management strategies involving the movement of deer, and would deprive deer breeders of the ability to engage in the business of buying and selling breeder deer. Therefore, this alternative was rejected because the department determined that it placed an avoidable burden on the regulated community.<<<

 

Circulation of prions within dust on a scrapie affected farm

 

Kevin C Gough1, Claire A Baker2, Hugh A Simmons3, Steve A Hawkins3 and Ben C Maddison2*

 

Abstract

 

Prion diseases are fatal neurological disorders that affect humans and animals. Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk are contagious prion diseases where environmental reservoirs have a direct link to the transmission of disease. Using protein misfolding cyclic amplification we demonstrate that scrapie PrPSc can be detected within circulating dusts that are present on a farm that is naturally contaminated with sheep scrapie. The presence of infectious scrapie within airborne dusts may represent a possible route of infection and illustrates the difficulties that may be associated with the effective decontamination of such scrapie affected premises.

 

snip...

 

Discussion

 

We present biochemical data illustrating the airborne movement of scrapie containing material within a contaminated farm environment. We were able to detect scrapie PrPSc within extracts from dusts collected over a 70 day period, in the absence of any sheep activity. We were also able to detect scrapie PrPSc within dusts collected within pasture at 30 m but not at 60 m distance away from the scrapie contaminated buildings, suggesting that the chance of contamination of pasture by scrapie contaminated dusts decreases with distance from contaminated farm buildings. PrPSc amplification by sPMCA has been shown to correlate with infectivity and amplified products have been shown to be infectious [14,15]. These experiments illustrate the potential for low dose scrapie infectivity to be present within such samples. We estimate low ng levels of scrapie positive brain equivalent were deposited per m2 over 70 days, in a barn previously occupied by sheep affected with scrapie. This movement of dusts and the accumulation of low levels of scrapie infectivity within this environment may in part explain previous observations where despite stringent pen decontamination regimens healthy lambs still became scrapie infected after apparent exposure from their environment alone [16]. The presence of sPMCA seeding activity and by inference, infectious prions within dusts, and their potential for airborne dissemination is highly novel and may have implications for the spread of scrapie within infected premises. The low level circulation and accumulation of scrapie prion containing dust material within the farm environment will likely impede the efficient decontamination of such scrapie contaminated buildings unless all possible reservoirs of dust are removed. Scrapie containing dusts could possibly infect animals during feeding and drinking, and respiratory and conjunctival routes may also be involved. It has been demonstrated that scrapie can be efficiently transmitted via the nasal route in sheep [17], as is also the case for CWD in both murine models and in white tailed deer [18-20].

 

The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.

 

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4397813/pdf/13567_2015_Article_176.pdf

 

***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***

 

http://www.nature.com/articles/srep11573

 

***at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified.

 

P-145 Estimating chronic wasting disease resistance in cervids using real time quaking- induced conversion

 

Nicholas J Haley1, Rachel Rielinqer2, Kristen A Davenport3, W. David Walter4, Katherine I O'Rourke5, Gordon Mitchell6, Juergen A Richt2

 

1 Department of Microbiology and Immunology, Midwestern University, United States; 2Department of Diagnostic Medicine and Pathobiology, Kansas State University; 3Prion Research Center; Colorado State University; 4U.S. Geological Survey, Pennsylvania Cooperative Fish and Wildlife Research Unit; 5Agricultural Research Service, United States Department of Agriculture; 6Canadian Food Inspection Agency, National and OlE Reference Laboratory for Scrapie and CWO

 

In mammalian species, the susceptibility to prion diseases is affected, in part, by the sequence of the host's prion protein (PrP). In sheep, a gradation from scrapie susceptible to resistant has been established both in vivo and in vitro based on the amino acids present at PrP positions 136, 154, and 171, which has led to global breeding programs to reduce the prevalence of scrapie in domestic sheep. In cervids, resistance is commonly characterized as a delayed progression of chronic wasting disease (CWD); at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified. To model the susceptibility of various naturally-occurring and hypothetical cervid PrP alleles in vitro, we compared the amplification rates and efficiency of various CWD isolates in recombinant PrPC using real time quaking-induced conversion. We hypothesized that amplification metrics of these isolates in cervid PrP substrates would correlate to in vivo susceptibility - allowing susceptibility prediction for alleles found at 10 frequency in nature, and that there would be an additive effect of multiple resistant codons in hypothetical alleles. Our studies demonstrate that in vitro amplification metrics predict in vivo susceptibility, and that alleles with multiple codons, each influencing resistance independently, do not necessarily contribute additively to resistance. Importantly, we found that the white-tailed deer 226K substrate exhibited the slowest amplification rate among those evaluated, suggesting that further investigation of this allele and its resistance in vivo are warranted to determine if absolute resistance to CWD is possible.

 

***at present, no cervid PrP allele conferring absolute resistance to prion infection has been identified.

 

PRION 2016 CONFERENCE TOKYO

 

http://prion2016.org/dl/newsletter_03.pdf

 

*** Grant Agreement number: 222887 ***

 

*** Project acronym: PRIORITY ***

 

*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***

 

Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014

 

Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es

 

Project website¡Error! Marcador no definido. address: www.prionpriority.eu

 

PRIORITY, PROJECT FINAL REPORT

 

*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***

 

snip...

 

Block D: Prion epidemiology

 

Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.

 

In parallel, studies in shep have concluded that: 

 

*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes 

 

*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.

 

*** The combination of data from all our studies leads us to conclude that: 

 

*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice). 

 

*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.

 

snip...

 

We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:

 

a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:

 

• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;

 

• the uncertainties related to prion epidemiology in animal populations;

 

• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents;

 

• the intrinsic capacity of prions to cross interspecies transmission barriers;

 

• the lack of sensitive methodology for identifying cross contamination in food.

 

• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.

 

The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.

 

b. Atypical prion agents and surveillance

 

Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.

 

*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.

 

*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.

 

*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.

 

*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable

 

• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)

 

• to improve the sensitivity of detection assays that are applied in the field towards this type of agent

 

• to maintain a robust surveillance of both animal and human populations

 

c. The need for extended research on prions

 

Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.

 

Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.

 

Position of the Priority consortium

 

Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk

 

Origins of prion epidemies

 

Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.

 

In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.

 

The human prion problem

 

The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.

 

Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.

 

The animal prion problem

 

An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.

 

We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.

 

The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.

 

http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf

 

WS-01: Prion diseases in animals and zoonotic potential 2016

 

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

 

Taylor & Francis

 

Prion 2016 Animal Prion Disease Workshop Abstracts

 

WS-01: Prion diseases in animals and zoonotic potential

 

Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,

 

Natalia Fernandez-Borges a. and Alba Marin-Moreno a

 

"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France

 

Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.

 

To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.

 

These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.

 

Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 

http://www.tandfonline.com/na101/home/literatum/publisher/tandf/journals/content/kprn20/2016/kprn20.v010.sup01/19336896.2016.1163048/20160418/19336896.2016.1163048.fp.png_v03

 

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

 

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

 

http://scrapie-usa.blogspot.com/2016/04/scrapie-ws-01-prion-diseases-in-animals.html

 

P.86: Estimating the risk of transmission of BSE and scrapie to ruminants and humans by protein misfolding cyclic amplification

 

Morikazu Imamura, Naoko Tabeta, Yoshifumi Iwamaru, and Yuichi Murayama National Institute of Animal Health; Tsukuba, Japan

 

To assess the risk of the transmission of ruminant prions to ruminants and humans at the molecular level, we investigated the ability of abnormal prion protein (PrPSc) of typical and atypical BSEs (L-type and H-type) and typical scrapie to convert normal prion protein (PrPC) from bovine, ovine, and human to proteinase K-resistant PrPSc-like form (PrPres) using serial protein misfolding cyclic amplification (PMCA).

 

Six rounds of serial PMCA was performed using 10% brain homogenates from transgenic mice expressing bovine, ovine or human PrPC in combination with PrPSc seed from typical and atypical BSE- or typical scrapie-infected brain homogenates from native host species. In the conventional PMCA, the conversion of PrPC to PrPres was observed only when the species of PrPC source and PrPSc seed matched. However, in the PMCA with supplements (digitonin, synthetic polyA and heparin), both bovine and ovine PrPC were converted by PrPSc from all tested prion strains. On the other hand, human PrPC was converted by PrPSc from typical and H-type BSE in this PMCA condition.

 

*** Although these results were not compatible with the previous reports describing the lack of transmissibility of H-type BSE to ovine and human transgenic mice, our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. ***

 

O.08: H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism: Clinical and pathologic features in wild-type and E211K cattle following intracranial inoculation

 

S Jo Moore, M Heather West Greenlee, Jodi Smith, Eric Nicholson, Cathy Vrentas, and Justin Greenlee United States Department of Agriculture; Ames, IA USA

 

In 2006 an H-type bovine spongiform encephalopathy (BSE) case was reported in an animal with an unusual polymorphism (E211K) in the prion protein gene. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele are predisposed to rapid onset of H-type BSE when exposed. The purpose of this study was to investigate the phenotype of this BSE strain in wild-type (E211E) and E211K heterozygous cattle. One calf carrying the wild-type allele and one E211K calf were inoculated intracranially with H-type BSE brain homogenate from the US 2006 case that also carried one K211 allelle. In addition, one wild-type calf and one E211K calf were inoculated intracranially with brain homogenate from a US 2003 classical BSE case. All animals succumbed to clinical disease. Survival times for E211K H-type BSE inoculated catttle (10 and 18 months) were shorter than the classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Animals challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K H-type BSE and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains.

 

This study demonstrates that the phenotype of E211K H-type BSE remains stable when transmitted to cattle without the E211K polymorphism, and exhibits a number of features that differ from classical BSE in both wild-type and E211K cattle.

 

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

 

Emmanuel Comoy, Jacqueline Mikol, Val erie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France

 

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases).

 

Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.

 

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), is the third potentially zoonotic PD (with BSE and L-type BSE), thus questioning the origin of human sporadic cases.

 

*** We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.

 

P.73: Oral challenge of goats with atypical scrapie

 

Silvia Colussi1, Maria Mazza1, Francesca Martucci1, Simone Peletto1, Cristiano Corona1, Marina Gallo1, Cristina Bona1, Romolo Nonno2, Michele Di Bari2, Claudia D’Agostino2, Nicola Martinelli3, Guerino Lombardi3, and Pier Luigi Acutis1 1Istituto Zooprofilattico Sperimentale del Piemonte; Liguria e Valle d’Aosta; Turin, Italy; 2Istituto Superiore di Sanit a; Rome, Italy; 3Istituto Zooprofilattico Sperimentale della Lombardia e dell’Emilia Romagna; Brescia, Italy

 

Atypical scrapie transmission has been demonstrated in sheep by intracerebral and oral route (Simmons et al., Andreoletti et al., 2011) but data about goats are not available yet. In 2006 we orally challenged four goats, five months old, with genotype R/H and R/R at codon 154. Animals died starting from 24 to 77 months p.i. without clinical signs. They all resulted negative for scrapie in CNS and peripheral tissues using Western blot and immunohistochemistry. Nevertheless these goats could still represent carriers. This hypothesis was investigated through bioassay in tg338 mice, a sensitive animal model for atypical scrapie infectivity. By end-point dilution titration, the starting inoculum contained 106.8 ID50/g. In contrast, all tissues from challenged goats were negative by bioassay. These negative results could be explained with the low infectivity of the starting inoculum, which could have been unable to induce Prion 2015 Poster Abstracts S49 disease or infectivity within our period of observation. However the challenge conditions could have been a bias too: as the matter of the fact, while the oral challenge of classical scrapie is still effective in sheep 6–10 months old (Andreoletti et al., 2011), Simmons et al. (2011) demonstrated a very short efficacy period for atypical scrapie (24 hours after birth), hypothesizing that natural transmission could occur mainly via milk. Our work suggests that this could be true also for goats and it should be taken into account in oral challenges. However a low susceptibility of goats to atypical scrapie transmission via oral route cannot be excluded.

 

https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf

 

>>> These results suggest that (i) at the level of protein-protein interactions, CWD adapts to a new species more readily than does BSE and (ii) the barrier preventing transmission of CWD to humans may be less robust than estimated.

 

Accepted manuscript posted online 8 July 2015.

 

Insights into Chronic Wasting Disease and Bovine Spongiform Encephalopathy Species Barriers by Use of Real-Time Conversion

 

Kristen A. Davenport, Davin M. Henderson, Jifeng Bian, Glenn C. Telling, Candace K. Mathiason and Edward A. Hoover Prion Research Center, Department of Microbiology, Immunology and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, Colorado, USA K. L. Beemon, Editor + Author Affiliations

 

Next Section ABSTRACT The propensity for transspecies prion transmission is related to the structural characteristics of the enciphering and new host PrP, although the exact mechanism remains incompletely understood. The effects of variability in prion protein on cross-species prion transmission have been studied with animal bioassays, but the influence of prion protein structure versus that of host cofactors (e.g., cellular constituents, trafficking, and innate immune interactions) remains difficult to dissect. To isolate the effects of protein-protein interactions on transspecies conversion, we used recombinant PrPC and real-time quaking-induced conversion (RT-QuIC) and compared chronic wasting disease (CWD) and classical bovine spongiform encephalopathy (cBSE) prions. To assess the impact of transmission to a new species, we studied feline CWD (fCWD) and feline BSE (i.e., feline spongiform encephalopathy [FSE]). We cross-seeded fCWD and FSE into each species' full-length, recombinant PrPC and measured the time required for conversion to the amyloid (PrPRes) form, which we describe here as the rate of amyloid conversion. These studies revealed the following: (i) CWD and BSE seeded their homologous species' PrP best; (ii) fCWD was a more efficient seed for feline rPrP than for white-tailed deer rPrP; (iii) conversely, FSE more efficiently converted bovine than feline rPrP; (iv) and CWD, fCWD, BSE, and FSE all converted human rPrP, although not as efficiently as homologous sCJD prions. These results suggest that (i) at the level of protein-protein interactions, CWD adapts to a new species more readily than does BSE and (ii) the barrier preventing transmission of CWD to humans may be less robust than estimated.

 

IMPORTANCE We demonstrate that bovine spongiform encephalopathy prions maintain their transspecies conversion characteristics upon passage to cats but that chronic wasting disease prions adapt to the cat and are distinguishable from the original prion. Additionally, we showed that chronic wasting disease prions are effective at seeding the conversion of normal human prion protein to an amyloid conformation, perhaps the first step in crossing the species barrier.

 

snip...

 

Enciphering characteristics of cBSE and cBSE-derived prions are conserved after transspecies transmission.cBSE and CWD are prion diseases that have been naturally passaged in their respective species (cattle and deer), whereas feline spongiform encephalopathy (FSE) and feline chronic wasting disease (fCWD) are first-passage infections in a new host species (cat). To investigate the biochemical properties of cBSE and CWD after transspecies transmission to felines, we compared the amyloidogenicity of fCWD and FSE in the original host and in feline substrate. We found fCWD to be a more efficient seed for its new (feline) host, suggesting that adaptation to the new host had occurred (Fig. 4A). In contrast, FSE remained a more efficient seed for its enciphering (bovine) host, despite its derivation from feline brain PrPC (Fig. 4B). Thereby, these cross-species seeding experiments in RT-QuIC indicated that the characteristics of cBSE were maintained upon passage to a new species whereas CWD had adapted to its new host. These findings in felids suggest that cBSE may retain its ability to cross species barriers even after transmission to a new host species and that CWD may change substantially upon transspecies transmission.

 

Human rPrPC can be converted by bovine, feline, and cervid prions.The threat of zoonotic transmission of prion disease is evident and well documented, yet such transmission is uncommonly observed and incompletely understood. We thereby explored the propensity of heterologous prions to convert human rPrP. In these human rPrPC experiments, we used sporadic CJD brain as a positive control and normal bovine, white-tailed deer, and feline brain as negative controls. sCJD, as expected, seeded human rPrPC most efficiently, so all other seeds were normalized to the rate of conversion of sCJD. We found human rPrPC to be a competent substrate in RT-QuIC for CWD, fCWD, cBSE, and FSE (Fig. 5A). Interestingly, CWD and fCWD converted human rPrPC more efficiently than did cBSE and FSE. These data suggest that at the level of PrPC-PrP seed interaction, CWD has the ability to template the conversion of human rPrPC to ThT-positive amyloid. In order to assess whether CWD was faster than cBSE due to an increased concentration of prion seed, we performed Western blotting on the seed inocula. Western blots indicated that the cBSE sample had a higher concentration of PrPRes than the CWD sample, indicating that CWD was not a better seed than cBSE due to PrPRes content (Fig. 5B). Finally, we assessed the behavior of 8 CWD field isolates, brain samples from white-tailed deer infected naturally and verified to be positive using full-length white-tailed deer RT-QuIC (Fig. 5C). All 8 of these isolates converted human rPrPC, confirming that our observations were not due to the use of experimentally CWD (Fig. 5D). In all, these experiments suggest that the CWD prions naturally circulating in the western United States have the capacity to convert human rPrPC in this assay of protein-protein interactions.

 

snip...

 

In summary, real-time conversion demonstrates that CWD and BSE prions differ in their enciphering rigidity and plasticity across species barriers. One illustration is the conservation versus adaptation of enciphering prion characteristics upon passage to cats. These experiments also demonstrate that human rPrP can be converted to amyloid by both cBSE and CWD prions. These data point to the importance of deciphering the mechanisms by which prions infect and adapt to a new species and of prompt continued vigilance regarding indirect pathways that may facilitate transspecies prion transmission.

 

http://jvi.asm.org/content/89/18/9524.full

 

Monday, September 19, 2016

 

Evidence of scrapie transmission to sheep via goat milk

 

http://scrapie-usa.blogspot.com/2016/09/evidence-of-scrapie-transmission-to.html

 

CWD TSE PRION HUMAN ZOONOSIS POTENTIAL, has it already happened, and being masked as sporadic CJD? and what about iatrogenic, or the pass if forward, friendly fire mode of transmission of cwd to humans, same thing, sporadic cjd ?

 

*** WDA 2016 NEW YORK ***

 

We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions.

 

Student Presentations Session 2

 

The species barriers and public health threat of CWD and BSE prions

 

Ms. Kristen Davenport1, Dr. Davin Henderson1, Dr. Candace Mathiason1, Dr. Edward Hoover1 1Colorado State University

 

Chronic wasting disease (CWD) is spreading rapidly through cervid populations in the USA. Bovine spongiform encephalopathy (BSE, mad cow disease) arose in the 1980s because cattle were fed recycled animal protein. These and other prion diseases are caused by abnormal folding of the normal prion protein (PrP) into a disease causing form (PrPd), which is pathogenic to nervous system cells and can cause subsequent PrP to misfold. CWD spreads among cervids very efficiently, but it has not yet infected humans. On the other hand, BSE was spread only when cattle consumed infected bovine or ovine tissue, but did infect humans and other species. The objective of this research is to understand the role of PrP structure in cross-species infection by CWD and BSE. To study the propensity of each species’ PrP to be induced to misfold by the presence of PrPd from verious species, we have used an in vitro system that permits detection of PrPd in real-time. We measured the conversion efficiency of various combinations of PrPd seeds and PrP substrate combinations. We observed the cross-species behavior of CWD and BSE, in addition to feline-adapted CWD and BSE. We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. CWD is unique among prion diseases in its rapid spread in natural populations. BSE prions are essentially unaltered upon passage to a new species, while CWD adapts to the new species. This adaptation has consequences for surveillance of humans exposed to CWD.

 

Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders

 

 http://www.wda2016.org/uploads/5/8/6/1/58613359/wda_2016_conference_proceedings_low_res.pdf

 

 http://csu-cvmbs.colostate.edu/Documents/research-day-2016.pdf

 

PRION 2016 TOKYO

 

Zoonotic Potential of CWD Prions: An Update

 

Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3, Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6, Pierluigi Gambetti1, Qingzhong Kong1,5,6

 

1Department of Pathology, 3National Prion Disease Pathology Surveillance Center, 5Department of Neurology, 6National Center for Regenerative Medicine, Case Western Reserve University, Cleveland, OH 44106, USA.

 

4Department of Biological Sciences and Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada,

 

2Encore Health Resources, 1331 Lamar St, Houston, TX 77010

 

Chronic wasting disease (CWD) is a widespread and highly transmissible prion disease in free-ranging and captive cervid species in North America. The zoonotic potential of CWD prions is a serious public health concern, but the susceptibility of human CNS and peripheral organs to CWD prions remains largely unresolved. We reported earlier that peripheral and CNS infections were detected in transgenic mice expressing human PrP129M or PrP129V. Here we will present an update on this project, including evidence for strain dependence and influence of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of experimental human CWD prions.

 

PRION 2016 TOKYO

 

In Conjunction with Asia Pacific Prion Symposium 2016

 

PRION 2016 Tokyo

 

Prion 2016

 

 http://prion2016.org/dl/newsletter_03.pdf

 

Cervid to human prion transmission

 

Kong, Qingzhong

 

Case Western Reserve University, Cleveland, OH, United States

 

Abstract

 

Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that:

 

(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues;

 

(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence;

 

(3) Reliable essays can be established to detect CWD infection in humans;and

 

(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.

 

Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of "humanized" Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental "human CWD" samples will also be generated for Aim 3.

 

Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1.

 

Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental "human CWD" samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions.

 

Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans.

 

Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans.

 

 http://grantome.com/grant/NIH/R01-NS088604-01A1

 

 http://grantome.com/grant/NIH/R56-AI122273-01A1

 

 http://grantome.com/grant/NIH/R01-NS061902-07

 

LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$

 

*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***

 

 https://www.landesbioscience.com/journals/prion/article/28124/?nocache=112223249

 

PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS

 

*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***

 

O18

 

Zoonotic Potential of CWD Prions

 

Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1, Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy, 3Encore Health Resources, Houston, Texas, USA

 

*** These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.

 

==================

 

***These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.***

 

==================

 

P.105: RT-QuIC models trans-species prion transmission

 

Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA

 

Conversely, FSE maintained sufficient BSE characteristics to more efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was competent for conversion by CWD and fCWD.

 

***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.

 

================

 

***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.***

 

================

 

 https://prion2015.files.wordpress.com/2015/05/programguide1.pdf

 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

 http://wwwnc.cdc.gov/eid/article/20/1/13-0858_article.htm

 

 http://chronic-wasting-disease.blogspot.com/2014/01/molecular-barriers-to-zoonotic.html

 

*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***

 

 https://www.landesbioscience.com/journals/prion/article/28124/?nocache=112223249

 

*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

 

 http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf

 

***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********

 

CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994

 

Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)

 

These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...

 

Table 9 presents the results of an analysis of these data.

 

There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).

 

Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.

 

There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).

 

The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).

 

There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).

 

The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).

 

snip...

 

It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).

 

snip...

 

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...

 

snip...

 

In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)

 

snip...see full report ;

 

 http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf

 

CJD9/10022

 

October 1994

 

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

 

Dear Mr Elmhirst,

 

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

 

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

 

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

 

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

 

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

 

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

 

 http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

 

Monday, May 02, 2016

 

*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***

 

 http://chronic-wasting-disease.blogspot.com/2016/05/zoonotic-potential-of-cwd-prions-update.html

 

*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD

 

 http://chronic-wasting-disease.blogspot.com/2014/06/prion-2014-chronic-wasting-disease-cwd.html

 

*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent

 

*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,

 

*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.

 

PPo2-27:

 

Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions

 

*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.

 

PPo2-7:

 

Biochemical and Biophysical Characterization of Different CWD Isolates

 

*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.

 

 https://www.landesbioscience.com/journals/prion/Prion4-3-PPo2.pdf

 

Envt.07:

 

Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease

 

***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.

 

 http://www.landesbioscience.com/journals/prion/Prion5-Supp-PrionEnvironment.pdf?nocache=1333529975

 

>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<

 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

Wednesday, January 01, 2014

 

Molecular Barriers to Zoonotic Transmission of Prions

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

 http://wwwnc.cdc.gov/eid/article/20/1/13-0858_article.htm

 

 http://chronic-wasting-disease.blogspot.com/2014/01/molecular-barriers-to-zoonotic.html

 

Envt.07:

 

Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease

 

***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.

 

Yet, it has to be noted that our assessments of PrPTSE levels in skeletal muscles were based on findings in presumably pre- or subclinically infected animals. Therefore, the concentration of PrPTSE in skeletal muscles of WTD with clinically manifest CWD may possibly exceed our estimate which refers to clinically inconspicuous animals that are more likely to enter the human food chain. Our tissue blot findings in skeletal muscles from CWD-infected WTD would be consistent with an anterograde spread of CWD prions via motor nerve fibres to muscle tissue (figure 4A). Similar neural spreading pathways of muscle infection were previously found in hamsters orally challenged with scrapie [28] and suggested by the detection of PrPTSE in muscle fibres and muscle-associated nerve fascicles of clinically-ill non-human primates challenged with BSE prions [29]. Whether the absence of detectable PrPTSE in myofibers observed in our study is a specific feature of CWD in WTD, or was due to a pre- or subclinical stage of infection in the examined animals, remains to be established. In any case, our observations support previous findings suggesting the precautionary prevention of muscle tissue from CWD-infected WTD in the human diet, and highlight the need to comprehensively elucidate of whether CWD may be transmissible to humans. While the understanding of TSEs in cervids has made substantial progress during the past few years, the assessment and management of risks possibly emanating from prions in skeletal muscles of CWD-infected cervids requires further research.

 

 http://www.landesbioscience.com/journals/prion/Prion5-Supp-PrionEnvironment.pdf?nocache=1333529975

 

 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3069970/

 

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C. Angers1,*, Shawn R. Browning1,*,†, Tanya S. Seward2, Christina J. Sigurdson4,‡, Michael W. Miller5, Edward A. Hoover4, Glenn C. Telling1,2,3,§ + Author Affiliations

 

1 Department of Microbiology, Immunology and Molecular Genetics, University of Kentucky, Lexington, KY 40536, USA. 2 Sanders Brown Center on Aging, University of Kentucky, Lexington, KY 40536, USA. 3 Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4 Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5 Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA. ↵§ To whom correspondence should be addressed. E-mail: gtell2@uky.edu ↵* These authors contributed equally to this work.

 

↵† Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, FL 33458, USA.

 

↵‡ Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.

 

Abstract The emergence of chronic wasting disease (CWD) in deer and elk in an increasingly wide geographic area, as well as the interspecies transmission of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt Jakob disease, have raised concerns about the zoonotic potential of CWD. Because meat consumption is the most likely means of exposure, it is important to determine whether skeletal muscle of diseased cervids contains prion infectivity. Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.

 

 http://www.sciencemag.org/content/311/5764/1117.long

 

Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin Because It May Contain Meat Derived From An Elk Confirmed To Have Chronic Wasting Disease

 

Contact: Exotic Meats USA 1-800-680-4375

 

FOR IMMEDIATE RELEASE -- February 9, 2009 -- Exotic Meats USA of San Antonio, TX is initiating a voluntary recall of Elk Tenderloin because it may contain meat derived from an elk confirmed to have Chronic Wasting Disease (CWD). The meat with production dates of December 29, 30 and 31, 2008 was purchased from Sierra Meat Company in Reno, NV. The infected elk came from Elk Farm LLC in Pine Island, MN and was among animals slaughtered and processed at USDA facility Noah’s Ark Processors LLC.

 

Chronic Wasting Disease (CWD) is a fatal brain and nervous system disease found in elk and deer. The disease is caused by an abnormally shaped protein called a prion, which can damage the brain and nerves of animals in the deer family. Currently, it is believed that the prion responsible for causing CWD in deer and elk is not capable of infecting humans who eat deer or elk contaminated with the prion, but the observation of animal-to-human transmission of other prion-mediated diseases, such as bovine spongiform encephalopathy (BSE), has raised a theoretical concern regarding the transmission of CWD from deer or elk to humans. At the present time, FDA believes the risk of becoming ill from eating CWD-positive elk or deer meat is remote. However, FDA strongly advises consumers to return the product to the place of purchase, rather than disposing of it themselves, due to environmental concerns.

 

Exotic Meats USA purchased 1 case of Elk Tenderloins weighing 16.9 lbs. The Elk Tenderloin was sold from January 16 – 27, 2009. The Elk Tenderloins was packaged in individual vacuum packs weighing approximately 3 pounds each. A total of six packs of the Elk Tenderloins were sold to the public at the Exotic Meats USA retail store. Consumers who still have the Elk Tenderloins should return the product to Exotic Meats USA at 1003 NE Loop 410, San Antonio, TX 78209. Customers with concerns or questions about the Voluntary Elk Recall can call 1-800-680-4375. The safety of our customer has always been and always will be our number one priority.

 

Exotic Meats USA requests that for those customers who have products with the production dates in question, do not consume or sell them and return them to the point of purchase. Customers should return the product to the vendor. The vendor should return it to the distributor and the distributor should work with the state to decide upon how best to dispose. If the consumer is disposing of the product he/she should consult with the local state EPA office.

 

#

 

 http://www.fda.gov/Safety/Recalls/ArchiveRecalls/2009/ucm128543.htm

 

COLORADO: Farmer's market meat recalled after testing positive for CWD

 

24.dec.08 9News.com Jeffrey Wolf

 

Elk meat that was sold at a farmer's market is being recalled because tests show it was infected with chronic wasting disease. The Boulder County Health Department and Colorado Department of Public Health and Environment issued the recall Wednesday after the meat was sold at the Boulder County Fairgrounds on Dec. 13. Although there isn't any human health risk connected with CWD, the recalled was issued as a precaution. About 15 elk were bought from a commercial ranch in Colorado in early December and processed at a licensed plant. All 15 were tested for CWD and one came up positive. The labeling on the product would have the following information: *Seller: High Wire Ranch *The type of cut: "chuck roast," "arm roast," "flat iron," "ribeye steak," "New York steak," "tenderloin," "sirloin tip roast," "medallions" or "ground meat." *Processor: Cedaredge Processing *The USDA triangle containing the number "34645" People with questions about this meat can contact John Pape, epidemiologist at the Colorado Department of Public Health and Environment at 303-692-2628.

 

 http://www.9news.com/money/consumer/article.aspx?storyid=106536&catid=103

 

COULD NOT FIND any warning or recalls on these two sites confirming their recall of CWD infected meat. ...TSS

 

 http://www.bouldercounty.org/health/environ/foodsafety/index.htm

 

 http://www.cdphe.state.co.us/

 

Wednesday, April 06, 2011

 

Presence and Seeding Activity of Pathological Prion Protein (PrPTSE) in Skeletal Muscles of White-Tailed Deer Infected with Chronic Wasting Disease

 

 http://chronic-wasting-disease.blogspot.com/2011/04/presence-and-seeding-activity-of.html

 

Prion Infectivity in Fat of Deer with Chronic Wasting Disease

 

Brent Race,# Kimberly Meade-White,# Richard Race, and Bruce Chesebro* Rocky Mountain Laboratories, 903 South 4th Street, Hamilton, Montana 59840

 

Received 2 June 2009/ Accepted 24 June 2009

 

ABSTRACT Top ABSTRACT TEXT REFERENCES

 

Chronic wasting disease (CWD) is a neurodegenerative prion disease of cervids. Some animal prion diseases, such as bovine spongiform encephalopathy, can infect humans; however, human susceptibility to CWD is unknown. In ruminants, prion infectivity is found in central nervous system and lymphoid tissues, with smaller amounts in intestine and muscle. In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.

 

snip...

 

The highest risk of human contact with CWD might be through exposure to high-titer CNS tissue through accidental skin cuts or corneal contact at the time of harvest and butchering. However, the likelihood of a human consuming fat infected with a low titer of the CWD agent is much higher. It is impossible to remove all the fat present within muscle tissue, and fat consumption is inevitable when eating meat. Of additional concern is the fact that meat from an individual deer harvested by a hunter is typically consumed over multiple meals by the same group of people. These individuals would thus have multiple exposures to the CWD agent over time, which might increase the chance for transfer of infection.

 

In the Rocky Mountain region of North America, wild deer are subject to predation by wolves, coyotes, bears, and mountain lions. Although canines such as wolves and coyotes are not known to be susceptible to prion diseases, felines definitely are susceptible to BSE (9) and might also be infected by the CWD agent. Deer infected with the CWD agent are more likely to be killed by predators such as mountain lions (11). Peripheral tissues, including lymph nodes, muscle, and fat, which harbor prion infectivity are more accessible for consumption than CNS tissue, which has the highest level of infectivity late in disease. Therefore, infectivity in these peripheral tissues may be important in potential cross-species CWD transmissions in the wild.

 

The present finding of CWD infectivity in deer fat tissue raises the possibility that prion infectivity might also be found in fat tissue of other infected ruminants, such as sheep and cattle, whose fat and muscle tissues are more widely distributed in both the human and domestic-animal food chains. Although the infectivity in fat tissues is low compared to that in the CNS, there may be significant differences among species and between prion strains. Two fat samples from BSE agent-infected cattle were reported to be negative by bioassay in nontransgenic RIII mice (3, 6). However, RIII mice are 10,000-fold-less sensitive to BSE agent infection than transgenic mice expressing bovine PrP (4). It would be prudent to carry out additional infectivity assays on fat from BSE agent-infected cattle and scrapie agent-infected sheep using appropriate transgenic mice or homologous species to determine the risk from these sources.

 

 http://jvi.asm.org/cgi/content/full/83/18/9608

 

0C7.04

 

North American Cervids Harbor Two Distinct CWD Strains

 

Authors

 

Angers, R. Seward, T, Napier, D., Browning, S., Miller, M., Balachandran A., McKenzie, D., Hoover, E., Telling, G. 'University of Kentucky; Colorado Division of Wildlife, Canadian Food Inspection Agency; University Of Wisconsin; Colorado State University.

 

Content

 

Despite the increasing geographic distribution and host range of CWD, little is known about the prion strain(s) responsible for distinct outbreaks of the disease. To address this we inoculated CWD-susceptible Tg(CerPrP)1536+/· mice with 29 individual prion samples from various geographic locations in North America. Upon serial passage, intrastudy incubation periods consistently diverged and clustered into two main groups with means around 210 and 290 days, with corresponding differences in neuropathology. Prion strain designations were utilized to distinguish between the two groups: Type I CWD mice succumbed to disease in the 200 day range and displayed a symmetrical pattern of vacuolation and PrPSc deposition, whereas Type II CWD mice succumbed to disease near 300 days and displayed a strikingly different pattern characterized by large local accumulations of florid plaques distributed asymmetrically. Type II CWD bears a striking resemblance to unstable parental scrapie strains such as 87A which give rise to stable, short incubation period strains such as ME7 under certain passage conditions. In agreement, the only groups of CWD-inoculated mice with unwavering incubation periods were those with Type I CWD. Additionally, following endpoint titration of a CWD sample, Type I CWD could be recovered only at the lowest dilution tested (10-1), whereas Type II CWD was detected in mice inoculated with all dilutions resulting in disease. Although strain properties are believed to be encoded in the tertiary structure of the infectious prion protein, we found no biochemical differences between Type I and Type II CWD. Our data confirm the co·existence of two distinct prion strains in CWD-infected cervids and suggest that Type II CWD is the parent strain of Type I CWD.

 

see page 29, and see other CWD studies ;

 

 http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf

 

Sunday, November 23, 2008

 

PRION October 8th - 10th 2008 Book of Abstracts

 

 http://bse-atypical.blogspot.com/2008/11/prion-october-8th-10th-2008-book-of.html

 

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research

 

2015 Annual Report

 

1a. Objectives (from AD-416): 1. Investigate the pathobiology of atypical transmissible spongiform encephalopathies (TSEs) in natural hosts. A. Investigate the pathobiology of atypical scrapie. B. Investigate the pathobiology of atypical bovine spongiform encephalopathy (BSE). 2. Investigate the horizontal transmission of TSEs. A. Assess the horizontal transmission of sheep scrapie in the absence of lambing. B. Determine routes of transmission in chronic wasting disease (CWD) infected premises. C. Assess oral transmission of CWD in reindeer. 3. Investigate determinants of CWD persistence. A. Determine CWD host range using natural routes of transmission. B. Investigate the pathobiology of CWD.

 

1b. Approach (from AD-416): The studies will focus on three animal transmissible spongiform encephalopathy (TSE) agents found in the United States: bovine spongiform encephalopathy (BSE); scrapie of sheep and goats; and chronic wasting disease (CWD) of deer, elk, and moose. The research will address sites of accumulation, routes of infection, environmental persistence, and ante mortem diagnostics with an emphasis on controlled conditions and natural routes of infection. Techniques used will include clinical exams, histopathology, immunohistochemistry and biochemical analysis of proteins. The enhanced knowledge gained from this work will help mitigate the potential for unrecognized epidemic expansions of these diseases in populations of animals that could either directly or indirectly affect food animals.

 

3. Progress Report: Research efforts directed toward meeting objective 1 of our project plan include work in previous years starting with the inoculation of animals for studies designed to address the pathobiology of atypical scrapie, atypical bovine spongiform encephalopathy (BSE), as well as a genetic version of BSE. Post-mortem examination of the animals inoculated with atypical scrapie has been initiated and laboratory analysis of the tissues is ongoing. Atypical BSE animals have developed disease and evaluation of the samples is currently underway. Animals inoculated with a genetic version of BSE have developed disease with a manuscript reporting these results was published (2012), and additional laboratory comparisons of genetic BSE to atypical and classical BSE are ongoing. In addition, we have investigated the possibility that atypical scrapie was present earlier than previously detected in the national flock by analyzing archived field isolates using methods that were unavailable at the time of original diagnosis. Sample quality was sufficiently degraded that modern methods, beyond those applied to the tissues at the time the tissues were archived, were not suitable for evaluation. In research pertaining to objective 2, "Investigate the horizontal transmission of TSEs", we have initiated a study to determine if cohousing non-lambing scrapie inoculated sheep is sufficient to transmit scrapie to neonatal lambs. At this time, scrapie free ewes have lambed in the presence of scrapie inoculated animals and the lambs are cohoused with these inoculated animals.

 

4. Accomplishments 1. Changes in retinal function in cattle can be used to identify different types of bovine spongiform encephalopathy (BSE). BSE belongs to a group of fatal, transmissible protein misfolding diseases known as transmissible spongiform encephalopathies (TSEs). Like other protein misfolding diseases including Parkinson's disease and Alzheimer's disease, TSEs are generally not diagnosed until the onset of disease after the appearance of unequivocal clinical signs. As such, identification of the earliest clinical signs of disease may facilitate diagnosis. The retina is the most accessible part of the central nervous system. ARS scientist in Ames IA described antemortem changes in retinal function and thickness that are detectable in BSE inoculated animals up to 11 months prior to the appearance of any other signs of clinical disease. Differences in the severity of these clinical signs reflect the amount of PrPSc accumulation in the retina and the resulting inflammatory response of the tissue. These results are the earliest reported clinical signs associated with TSE infection and provide a basis for understanding the pathology and evaluating therapeutic interventions. Further, this work shows that High-type BSE and classical BSE can be differentiated by eye examination alone, the first time BSE strains have been differentiable in a live animal.

 

2. Sheep genetics influences the susceptibility of sheep to scrapie. Sheep scrapie is a transmissible spongiform encephalopathy that can be transmitted between affected animals resulting in significant economic losses in affected flocks. The prion protein gene (PRNP) profoundly influences the susceptibility of sheep to the scrapie agent and the tissue levels and distribution of PrPSc in affected sheep. In this study, sheep of 3 different prion genetic types (denoted VRQ/VRQ, VRQ/ARR and ARQ/ARR) were inoculated and subsequently euthanized upon onset of disease. Disease aspects were uniform across genotypes and consistent with manifestations of classical scrapie. Mean survival time differences were associated with the genetic type such that VRQ/VRQ sheep survived 18 months, whereas VRQ/ARR and ARQ/ARR sheep survived 60 and 56 months, respectively. Microscopic evaluation revealed similar accumulations in central nervous system tissues regardless of host genetic type. PrPSc in lymphoid tissue was consistently abundant in VRQ/VRQ, present but confined to tonsil or retropharyngeal lymph node in 4/5 VRQ/ARR, and totally absent in ARQ/ARR sheep. The results of this study demonstrate the susceptibility of sheep with the ARQ/ARR genotype to scrapie by the intracranial inoculation route with PrPSc accumulation in CNS tissues, but prolonged incubation times and lack of PrPSc in lymphoid tissue. These results are important for science based policy with regard to testing of sheep for scrapie where some live animal testing is conducted using lymphoid tissues which would not detect scrapie in some specific genetic types which could limit the national scrapie eradication program.

 

Review Publications Greenlee J.J. 2014. The prion diseases of animals. In: McManus, L.M., Mitchell, R.N., editors. Pathobiology of Human Disease. San Diego: Elsevier. p. 1124-1133. Greenlee, J.J., Kunkle, R.A., Richt, J.A., Nicholson, E.M., Hamir, A.N. 2014. Lack of prion accumulation in lymphoid tissues of PRNP ARQ/ARR sheep intracranially inoculated with the agent of scrapie. PLoS One. 9(9):e108029. Greenlee, J.J., West Greenlee, M.,H. 2015. The transmissible spongiform encephalopathies of livestock. ILAR Journal. 56(1):7-25. Munoz-Gutierrez, J.F., Schneider, D.A., Baszler, T.V., Dinkel, K.D., Greenlee, J.J., Nicholson, E.M., Stanton, J.J. 2015. hTERT-immortalized ovine microglia propagate natural scrapie isolates. Virus Research. 198:35-43. Nicholson, E.M. 2015. Detection of the disease-associated form of the prion protein in biological samples. Bioanalysis. 7(2):253-261. West Greenlee, M.H., Smith, J.D., Platt, E.M., Juarez, J.R., Timms, L.L, Greenlee, J.J. 2015. Changes in retinal function and morphology are early clinical signs of disease in cattle with bovine spongiform encephalopathy. PLoS ONE. 10(3):e0119431. Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E., Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C., Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J. 2015. Transmission of scrapie prions to primate after an extended silent incubation period. Scientific Reports. 5:11573.

 

https://www.ars.usda.gov/research/project/?accnNo=421870&fy=2015

 

 Subject: Wisconsin Governor Scott Walker's DNR et al have floundered again on taking any actions on CWD TSE Prion disease, decides to put off now until March 2017

 

see past history of CWD in Wisconsin here ;

 

Thursday, September 29, 2016

 

Wisconsin Governor Scott Walker's DNR et al have floundered again on taking any actions on CWD TSE Prion disease, decides to put off now until March 2017

 

http://chronic-wasting-disease.blogspot.com/2016/09/wisconsin-governor-scott-walkers-dnr-et.html

 

Friday, September 30, 2016

 

Wisconsin CWD-positive white-tailed deer found on Oconto County hunting preserve Date: September 30, 2016

 

http://chronic-wasting-disease.blogspot.com/2016/09/wisconsin-cwd-positive-white-tailed.html

 

Saturday, July 09, 2016

 

Texas Intrastate – within state movement of all Cervid or Trucking Chronic Wasting Disease CWD TSE Prion Moratorium

 

http://chronic-wasting-disease.blogspot.com/2016/07/texas-intrastate-within-state-movement.html

 

Friday, July 01, 2016

 

*** TEXAS Thirteen new cases of chronic wasting disease (CWD) were confirmed at a Medina County captive white-tailed deer breeding facility on June 29, 2016***

 

http://chronic-wasting-disease.blogspot.com/2016/07/texas-thirteen-new-cases-of-chronic.html

 

Monday, July 18, 2016

 

Texas Parks Wildlife Dept TPWD HIDING TSE (CWD) in Deer Herds, Farmers Sampling Own Herds, Rapid Testing, False Negatives, a Recipe for Disaster

 

http://chronic-wasting-disease.blogspot.com/2016/07/texas-parks-wildlife-dept-tpwd-hiding.html

 

Wednesday, September 21, 2016

 

TAHC Passes Authorized Personnel Rule at 396th Commission Meeting “Certified CWD Sample Collector” to “Certified CWD Postmortem Sample Collector”

 

http://chronic-wasting-disease.blogspot.com/2016/09/tahc-passes-authorized-personnel-rule.html

 

Wednesday, September 28, 2016

 

TPWD CWD Sample Collector Trainings in the Trans Pecos and Panhandle

 

http://chronic-wasting-disease.blogspot.com/2016/09/tpwd-cwd-sample-collector-trainings-in.html

 

Thursday, September 22, 2016

 

TVMDL offers a new, faster CWD testing option

 

http://chronic-wasting-disease.blogspot.com/2016/09/tvmdl-offers-new-faster-cwd-testing.html

 

Wednesday, September 21, 2016

 

TAHC Passes Authorized Personnel Rule at 396th Commission Meeting “Certified CWD Sample Collector” to “Certified CWD Postmortem Sample Collector”

 

http://chronic-wasting-disease.blogspot.com/2016/09/tahc-passes-authorized-personnel-rule.html

 

Monday, July 18, 2016

 

Texas Parks Wildlife Dept TPWD HIDING TSE (CWD) in Deer Herds, Farmers Sampling Own Herds, Rapid Testing, False Negatives, a Recipe for Disaster

 

http://chronic-wasting-disease.blogspot.com/2016/07/texas-parks-wildlife-dept-tpwd-hiding.html

 

Wednesday, July 22, 2015

 

Texas Certified Chronic Wasting Disease CWD Sample Collector, like the Wolf Guarding the Henhouse

 

http://chronic-wasting-disease.blogspot.com/2015/07/texas-certified-chronic-wasting-disease.html

 

Just got off the phone with TAHC, and I wanted to confirm this. but it seems true, that in the state of Texas, even if you are a Captive game farmer, breeder, part of the captive industry at all, if you want to sample your own cervid for cwd, instead of the TAHC, TPWD, or Doctor, all you have to do is pass the Certified CWD Sample Collector course, and bingo, you sample your own herd. ...tss

 

Thursday, May 02, 2013

 

Chronic Wasting Disease (CWD) Texas Important Update on OBEX ONLY TESTING

 

http://chronic-wasting-disease.blogspot.com/2013/05/chronic-wasting-disease-cwd-texas.html

 

Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie Experiment 1964

 

How Did CWD Get Way Down In Medina County, Texas?

 

Confucius ponders...

 

Could the Scrapie experiments back around 1964 at Moore Air Force near Mission, Texas, could this area have been ground zero for CWD TSE Prion (besides the CWD cases that have waltzed across the Texas, New Mexico border near WSMR Trans Pecos region since around 2001)?

 

Epidemiology of Scrapie in the United States 1977

 

snip...

 

Scrapie Field Trial Experiments Mission, Texas

 

A Scrapie Field Trial was developed at Mission, Texas, to provide additional information for the eradication program on the epidemiology of natural scrapie. The Mission Field Trial Station is located on 450 acres of pastureland, part of the former Moore Air Force Base, near Mission, Texas. It was designed to bring previously exposed, and later also unexposed, sheep or goats to the Station and maintain and breed them under close observation for extended periods to determine which animals would develop scrapie and define more closely the natural spread and other epidemiological aspects of the disease.

 

The 547 previously exposed sheep brought to the Mission Station beginning in 1964 were of the Cheviot, Hampshire, Montadale, or Suffolk breeds. They were purchased as field outbreaks occurred, and represented 21 bloodlines in which scrapie had been diagnosed. Upon arrival at the Station, the sheep were maintained on pasture, with supplemental feeding as necessary. The station was divided into 2 areas: (1) a series of pastures and-pens occupied by male animals only, and (2) a series of pastures and pens occupied by females and young progeny of both sexes. ...

 

snip...see full text ;

 

http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf

 

Thursday, June 09, 2016

 

Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie TSE Prion Experiment 1964

 

How Did CWD Get Way Down In Medina County, Texas?

 

http://chronic-wasting-disease.blogspot.com/2016/06/how-did-cwd-get-way-down-in-medina.html

 

http://scrapie-usa.blogspot.com/2016/06/scrapie-field-trial-experiments-mission.html

 

Friday, April 22, 2016

 

*** Texas Scrapie Confirmed in a Hartley County Sheep where CWD was detected in a Mule Deer

 

http://scrapie-usa.blogspot.com/2016/04/texas-scrapie-confirmed-in-hartley.html

 

Friday, February 26, 2016

 

TEXAS Hartley County Mule Deer Tests Positive for Chronic Wasting Disease CWD TSE Prion

 

http://chronic-wasting-disease.blogspot.com/2016/02/texas-hartley-county-mule-deer-tests.html

 

I understand that the 84th Legislation might have made some terrible mistakes with regards to Chronic Wasting Disease CWD TSE Prion aka mad cow type disease, by weakening CWD rules for breeders.

 

Sunday, December 14, 2014

 

TEXAS 84th Legislature commencing this January, deer breeders are expected to advocate for bills that will seek to further deregulate their industry

 

http://chronic-wasting-disease.blogspot.com/2014/12/texas-84th-legislature-commencing-this.html

 

Tuesday, December 16, 2014

 

Texas 84th Legislature 2015 H.R. No. 2597 Kuempel Deer Breeding Industry TAHC TPWD CWD TSE PRION

 

 http://chronic-wasting-disease.blogspot.com/2014/12/texas-84th-legislature-2015-hr-no-2597.html

 

Monday, February 11, 2013

 

TEXAS CHRONIC WASTING DISEASE CWD Four New Positives Found in Trans Pecos

 

 http://chronic-wasting-disease.blogspot.com/2013/02/texas-chronic-wasting-disease-cwd-four.html

 

Tuesday, July 10, 2012

 

Chronic Wasting Disease Detected in Far West Texas

 

http://chronic-wasting-disease.blogspot.com/2012/07/chronic-wasting-disease-detected-in-far.html

 

Monday, March 26, 2012

 

Texas Prepares for Chronic Wasting Disease CWD Possibility in Far West Texas

 

http://chronic-wasting-disease.blogspot.com/2012/03/texas-prepares-for-chronic-wasting.html

 

2011 – 2012

 

Friday, October 28, 2011

 

CWD Herd Monitoring Program to be Enforced Jan. 2012 TEXAS

 

Greetings TAHC et al,

 

A kind greetings from Bacliff, Texas.

 

In reply to ;

 

Texas Animal Health Commission (TAHC) Announcement October 27, 2011

 

I kindly submit the following ;

 

http://chronic-wasting-disease.blogspot.com/2011/10/cwd-herd-monitoring-program-to-be.html

 

http://chronic-wasting-disease.blogspot.com/2013/02/texas-chronic-wasting-disease-cwd-four.html

 

***for anyone interested, here is some history of CWD along the Texas, New Mexico border, and my attempt to keep up with it...terry

 

snip...

 

see history CWD Texas, New Mexico Border ;

 

Monday, March 26, 2012

 

3 CASES OF CWD FOUND NEW MEXICO MULE DEER SEVERAL MILES FROM TEXAS BORDER

 

http://chronic-wasting-disease.blogspot.com/2012/03/3-cases-of-cwd-found-new-mexico-mule.html

 

Sunday, October 04, 2009

 

CWD NEW MEXICO SPREADING SOUTH TO TEXAS 2009 2009 Summary of Chronic Wasting Disease in New Mexico New Mexico Department of Game and Fish

 

http://chronic-wasting-disease.blogspot.com/2009/10/cwd-new-mexico-spreading-south-to-texas.html

 

*** Thursday, September 22, 2016

 

*** New Mexico CWD confirmed in 5 McGregor Range deer during the 2015-16 hunting season ***

 

http://chronic-wasting-disease.blogspot.com/2016/09/new-mexico-cwd-confirmed-in-5-mcgregor.html

 

Monday, August 29, 2016

 

*** NWHC USGS CHRONIC WASTING DISEASE CWD TSE PRION UPDATE

 

http://chronic-wasting-disease.blogspot.com/2016/08/nwhc-usgs-chronic-wasting-disease-cwd.html

 

http://chronic-wasting-disease.blogspot.com/

 

Thursday, August 18, 2016

 

*** PROCEEDINGS ONE HUNDRED AND Nineteenth ANNUAL MEETING of the USAHA BSE, CWD, SCRAPIE, PORCINE TSE PRION October 22 28, 2015 ***

 

http://chronic-wasting-disease.blogspot.com/2016/08/proceedings-one-hundred-and-nineteenth.html

 

Saturday, December 12, 2015

 

NOTICE: Environmental Impact Statement on Large Livestock Carcasses TSE Prion REPORT December 14, 2015

 

http://transmissiblespongiformencephalopathy.blogspot.com/2015/12/notice-environmental-impact-statement.html

 

Friday, August 14, 2015

 

Carcass Management During a Mass Animal Health Emergency Draft Programmatic Environmental Impact Statement—August 2015

 

http://transmissiblespongiformencephalopathy.blogspot.com/2015/08/carcass-management-during-mass-animal.html

 

***re-DEFRA What is the risk of a cervid TSE being introduced from Norway into Great Britain? Qualitative Risk Assessment September 2016***

 

***The likelihood of non-ruminant feed or cervid origin POAO being fed accidentally to reindeer or other captive deer in GB is very low.

 

***Therefore, assuming this ban is adhered to correctly the risk of farmed deer being exposed to animal feed containing deer protein from Norway is considered negligible but with associated uncertainty.

 

***However, given that non-ruminant feed produced elsewhere (eg the USA) may contain deer and moose PAP, it is theoretically possible that wild deer may be exposed to deer protein in legally imported non-ruminant feed. For this to occur, wild deer would need to access non-ruminant feed (e.g. pig, fish and chicken feed) on farms near their habitat. Alternatively, wild deer may be exposed to TSE prion in the faeces of pets that have consumed and digested imported, contaminated pet feed. Also to be considered is that food may be diverted to composting and the resulting effluate spread on pasture. The frequency in which these routes may occur is unknown and is considered to be a greater than negligible risk with associated uncertainty.***

 

 ================

 

PLEASE SEE ;

 

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II

 

___________________________________

 

PRODUCT

 

Bulk cattle feed made with recalled Darling’s 85% Blood Meal, Flash Dried, Recall # V-024-2007

 

CODE

 

Cattle feed delivered between 01/12/2007 and 01/26/2007

 

RECALLING FIRM/MANUFACTURER

 

Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007. Firm initiated recall is ongoing.

 

REASON

 

Blood meal used to make cattle feed was recalled because it was cross-contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

 

VOLUME OF PRODUCT IN COMMERCE

 

42,090 lbs.

 

DISTRIBUTION

 

WI

 

___________________________________

 

PRODUCT

 

Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot-Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI – 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J – PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A-BYPASS ML W/SMARTA, Recall # V-025-2007

 

CODE

 

The firm does not utilize a code - only shipping documentation with commodity and weights identified.

 

RECALLING FIRM/MANUFACTURER

 

Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete.

 

REASON

 

Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

 

VOLUME OF PRODUCT IN COMMERCE

 

9,997,976 lbs.

 

DISTRIBUTION

 

ID and NV

 

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

 

###

 

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm120446.htm

 

16 years post mad cow feed ban August 1997

 

2013

 

Sunday, December 15, 2013

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 

 http://madcowusda.blogspot.com/2013/12/fda-part-589-substances-prohibited-from.html

 

17 years post mad cow feed ban August 1997

 

Tuesday, December 23, 2014

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION

 

 http://madcowusda.blogspot.com/2014/12/fda-part-589-substances-prohibited-from.html

 

*** Monday, October 26, 2015 ***

 

*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE October 2015 ***

 

 http://madcowusda.blogspot.com/2015/10/fda-part-589-substances-prohibited-from.html

 

Thursday, July 24, 2014

 

*** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigations

 

 http://bse-atypical.blogspot.com/2014/07/protocol-for-further-laboratory.html

 

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

 

 http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143

 

SPECIFIED RISK MATERIAL SRM

 

Monday, June 20, 2016

 

*** Specified Risk Materials SRMs BSE TSE Prion Program ***

 

http://specifiedriskmaterial.blogspot.com/2016/06/specified-risk-materials-srms-bse-tse.html

 

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.

 

*** It also suggests a similar cause or source for atypical BSE in these countries.

 

http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf

 

Maximizing profits is all that is going on now, thanks to the OIE BSE MRR policy, the legal trading of all strains of TSE prion disease globally. ...Terry S. Singeltary Sr.

 

atypical BSE spontaneous sporadic ???

 

Saturday, May 26, 2012

 

Are USDA assurances on mad cow case 'gross oversimplification'?

 

SNIP...

 

*** What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”

 

*** The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health.

 

*** "(The agency) has no foundation on which to base that statement.”

 

*** “We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State.

 

*** In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said

 

*** The argument about feed is critical because if feed is the cause, not a spontaneous mutation, the California cow could be part of a larger outbreak.

 

SNIP...

 

http://bseusa.blogspot.com/2012/05/are-usda-assurances-on-mad-cow-case.html

 

Saturday, July 23, 2016

 

*** BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016

 

http://bovineprp.blogspot.com/2016/07/bovine-spongiform-encephalopathy-bse.html

 

Tuesday, July 26, 2016

 

*** Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016

 

http://bse-atypical.blogspot.com/2016/07/atypical-bovine-spongiform.html

 

 To further complicate things, we now know that science has shown that plants and vegetables can uptake the TSE Prion, and that the Scrapie agent can still be infectious from soil 16 years later. a frightening thought with the CWD running rampant now in North America (please see source reference materials below).

 

IF we don't do this, we have failed, and the TSE Prion agent will continue to spread, as it is doing as we speak.

 

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids, as well as non-ruminants such as cats and dogs as well, as soon as possible for the following reasons...

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

see Singeltary comment ;

 

 https://www.regulations.gov/document?D=FDA-2003-D-0432-0011

 

Saturday, January 31, 2015

 

European red deer (Cervus elaphus elaphus) are susceptible to Bovine Spongiform Encephalopathy BSE by Oral Alimentary route

 

http://transmissiblespongiformencephalopathy.blogspot.com/2015/01/european-red-deer-cervus-elaphus.html

 

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids as soon as possible for the following reasons...

 

======

 

In the USA, under the Food and Drug Administrations BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system.

 

***However, this recommendation is guidance and not a requirement by law.

 

======

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

see Singeltary comment ;

 

http://www.plosone.org/annotation/listThread.action?root=85351

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

https://www.regulations.gov/#!documentDetail;D=FDA-2003-D-0432-0011

 

Comment View document:Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards. Singeltary Comment,

 

Greetings FDA et al,

 

I would kindly like to comment on ;

 

Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards.

 

I implore that we close the mad cow feed loopholes with cervid, and we must enforce existing feed regulations against the BSE TSE Prion. we have failed terribly in this.

 

the august 1997 mad cow feed ban was nothing but ink on paper, imo. please see ;

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

see Singeltary comment ;

 

http://www.plosone.org/annotation/listThread.action?root=85351

 

SEE WHAT DEFRA MAFF ET AL SAID JUST LAST MONTH ABOUT THIS ;

 

Thursday, April 07, 2016

 

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 

SNIP...

 

https://www.gov.uk/government/uploads/sy...ing-disease.pdf

 

Summary and MORE HERE ;

 

What is the risk of chronic wasting disease being introduced into Great Britain? An updated Qualitative Risk Assessment March 2016

 

http://chronic-wasting-disease.blogspot.com/2016/04/what-is-risk-of-chronic-wasting-disease.html

 

 https://www.regulations.gov/document?D=FDA-2013-N-0764-0008

 

http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

Greetings again FDA and Mr. Pritchett et al,

 

I would kindly like to comment on ;

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

#158

 

Guidance for Industry

 

Use of Material from Deer and Elk in Animal Feed

 

This version of the guidance replaces the version made available September15, 2003.

 

This document has been revised to update the docket number, contact information, and standard disclosures. Submit comments on this guidance at any time.

 

Submit electronic comments to http://www.regulations.gov. Submit written comments to the Division of Dockets Management (HFA-305), Food and Drug Administration, 5630 Fishers Lane, Rm. 1061, Rockville, MD 20852. All comments should be identified with the Docket No. FDA-2003-D-0432 (formerly 03D-0186).

 

For further information regarding this guidance, contact Burt Pritchett, Center for Veterinary Medicine (HFV-222), Food and Drug Administration, 7519 Standish Place, Rockville, MD 20855, 240-402-6276, E-mail: burt.pritchett@fda.hhs.gov.

 

Additional copies of this guidance document may be requested from the Policy and Regulations Staff (HFV-6), Center for Veterinary Medicine, Food and Drug Administration, 7519 Standish Place, Rockville, MD 20855, and may be viewed on the Internet at either http://www.fda.gov/AnimalVeterinary/default.htm or http://www.regulations.gov.

 

U.S. Department of Health and Human Services Food and Drug Administration Center for Veterinary Medicine March 2016

 

Contains Nonbinding Recommendations

 

2

 

Guidance for Industry Use of Material from Deer and Elk in Animal Feed

 

This guidance represents the current thinking of the Food and Drug Administration (FDA or Agency) on this topic. It does not establish any rights for any person and is not binding on FDA or the public. You can use an alternative approach if it satisfies the requirements of the applicable statutes and regulations. To discuss an alternative approach, contact the FDA office responsible for this guidance as listed on the title page.

 

I. Introduction

 

Under FDA’s BSE feed regulation (21 CFR 589.2000) most material from deer and elk is prohibited for use in feed for ruminant animals. This guidance document describes FDA’s recommendations regarding the use in all animal feed of all material from deer and elk that are positive for Chronic Wasting Disease (CWD) or are considered at high risk for CWD. The potential risks from CWD to humans or non-cervid animals such as poultry and swine are not well understood. However, because of recent recognition that CWD is spreading rapidly in white-tailed deer, and because CWD’s route of transmission is poorly understood, FDA is making recommendations regarding the use in animal feed of rendered materials from deer and elk that are CWD-positive or that are at high risk for CWD.

 

In general, FDA’s guidance documents do not establish legally enforceable responsibilities. Instead, guidances describe the Agency’s current thinking on a topic and should be viewed only as recommendations, unless specific regulatory or statutory requirements are cited. The use of the word should in Agency guidances means that something is suggested or recommended, but not required.

 

II. Background

 

CWD is a neurological (brain) disease of farmed and wild deer and elk that belong in the animal family cervidae (cervids). Only deer and elk are known to be susceptible to CWD by natural transmission. The disease has been found in farmed and wild mule deer, white-tailed deer, North American elk, and in farmed black-tailed deer. CWD belongs to a family of animal and human diseases called transmissible spongiform encephalopathies (TSEs). These include bovine spongiform encephalopathy (BSE or “mad cow” disease) in cattle; scrapie in sheep and goats; and classical and variant Creutzfeldt-Jakob diseases (CJD and vCJD) in humans. There is no known treatment for these diseases, and there is no vaccine to prevent them. In addition, although validated postmortem diagnostic tests are available, there are no validated diagnostic tests for CWD that can be used to test for the disease in live animals.

 

Contains Nonbinding Recommendations

 

III. Use in animal feed of material from CWD-positive deer and elk

 

Material from CWD-positive animals may not be used in any animal feed or feed ingredients. Pursuant to Sec. 402(a)(5) of the Federal Food, Drug, and Cosmetic Act, animal feed and feed ingredients containing material from a CWD-positive animal would be considered adulterated. FDA recommends that any such adulterated feed or feed ingredients be recalled or otherwise removed from the marketplace.

 

IV. Use in animal feed of material from deer and elk considered at high risk for CWD Deer and elk considered at high risk for CWD include: (1) animals from areas declared by State officials to be endemic for CWD and/or to be CWD eradication zones; and (2) deer and elk that at some time during the 60-month period immediately before the time of slaughter were in a captive herd that contained a CWD-positive animal.

 

FDA recommends that materials from deer and elk considered at high risk for CWD no longer be entered into the animal feed system. Under present circumstances, FDA is not recommending that feed made from deer and elk from a non-endemic area be recalled if a State later declares the area endemic for CWD or a CWD eradication zone. In addition, at this time, FDA is not recommending that feed made from deer and elk believed to be from a captive herd that contained no CWD-positive animals be recalled if that herd is subsequently found to contain a CWD-positive animal.

 

V. Use in animal feed of material from deer and elk NOT considered at high risk for CWD FDA continues to consider materials from deer and elk NOT considered at high risk for CWD to be acceptable for use in NON-RUMINANT animal feeds in accordance with current agency regulations, 21 CFR 589.2000. Deer and elk not considered at high risk include: (1) deer and elk from areas not declared by State officials to be endemic for CWD and/or to be CWD eradication zones; and (2) deer and elk that were not at some time during the 60-month period immediately before the time of slaughter in a captive herd that contained a CWD-positive animal.

 

3

 

http://www.fda.gov/downloads/AnimalVeterinary/GuidanceComplianceEnforcement/GuidanceforIndustry/UCM052506.pdf?source=govdelivery&utm_medium=email&utm_source=govdelivery

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

Greetings again FDA and Mr. Pritchett et al,

 

MY comments and source reference of sound science on this very important issue are as follows ;

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

I kindly wish to once again submit to Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed.

 

Thank you kindly for allowing me to comment again, ...and again...and again, on a topic so important, why it is ‘NON-BINDING’ is beyond me.

 

this should have been finalized and made ‘BINDING’ or MANDATORY OVER A DECADE AGO.

 

but here lay the problem, once made ‘BINDING’ or ‘MANDATORY’, it is still nothing but ink on paper.

 

we have had a mad cow feed ban in place since August 1997, and since then, literally 100s of millions of pounds BANNED MAD COW FEED has been sent out to commerce and fed out (see reference materials).

 

ENFORCEMENT OF SAID BINDING REGULATIONS HAS FAILED US TOO MANY TIMES.

 

so, in my opinion, any non-binding or voluntary regulations will not work, and to state further, ‘BINDING’ or MANDATORY regulations will not work unless enforced.

 

with that said, we know that Chronic Wasting Disease CWD TSE Prion easily transmits to other cervid through the oral route.

 

the old transmission studies of BSE TSE floored scientist once they figured out what they had, and please don’t forget about those mink that were fed 95%+ dead stock downer cow, that all came down with TME. please see ;

 

It is clear that the designing scientists must also have shared Mr Bradleys surprise at the results because all the dose levels right down to 1 gram triggered infection.

 

http://web.archive.org/web/20061003022720/http://www.bseinquiry.gov.uk/files/ws/s145d.pdf

 

it is clear that the designing scientists must have also shared Mr Bradleys surprise at the results because all the dose levels right down to 1 gram triggered infection.

 

http://web.archive.org/web/20030526212610/http://www.bseinquiry.gov.uk/files/ws/s147f.pdf

 

Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle

 

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

 

snip...

 

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/mb/m09/tab05.pdf

 

To further complicate things, we now know that science has shown that plants and vegetables can uptake the TSE Prion, and that the Scrapie agent can still be infectious from soil 16 years later. a frightening thought with the CWD running rampant now in North America (please see source reference materials below).

 

IF we don’t not do this, we have failed, and the TSE Prion agent will continue to spread, as it is doing as we speak.

 

I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids, as well as non-ruminants such as cats and dogs as well, as soon as possible for the following reasons...

 

31 Jan 2015 at 20:14 GMT

 

*** Ruminant feed ban for cervids in the United States? ***

 

31 Jan 2015 at 20:14 GMT

 

see Singeltary comment ;

 

http://www.plosone.org/annotation/listThread.action?root=85351

 

please see further ;

 

REFERENCE MATERIALS

 

snip...

 

Terry S. Singeltary Sr.

 

Sunday, March 20, 2016

 

Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission

 

-------- Original Message --------

 

Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

 

Date: Fri, 16 May 2003 11:47:37 –0500

 

From: "Terry S. Singeltary Sr." mailto:flounder@wt.net

 

To: fdadockets@oc.fda.gov

 

Greetings FDA, i would kindly like to comment on; Docket 03D-0186FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability Several factors on this apparent voluntary proposal disturbs me greatly, please allow me to point them out;

 

snip...

 

Oral transmission and early lymphoid tropism of chronic wasting diseasePrPres in mule deer fawns (Odocoileus hemionus ) These results indicate that CWD PrP res can be detected in lymphoid tissues draining the alimentary tract within a few weeks after oral exposure to infectious prions and may reflect the initial pathway of CWD infection in deer. The rapid infection of deer fawns following exposure by the most plausible natural route is consistent with the efficient horizontal transmission of CWD in nature and enables accelerated studies of transmission and pathogenesis in the native species.

 

snip...

 

http://vir.sgmjournals.org/cgi/content/full/80/10/2757

 

now, just what is in that mad deer feed?

 

_ANIMAL PROTEIN_

 

Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES

 

Date: Sat, 25 May 2002 18:41:46 –0700

 

From: "Terry S. Singeltary Sr."

 

Reply-To: BSE-L

 

To: BSE-L

 

8420-20.5% Antler DeveloperFor Deer and Game in the wildGuaranteed Analysis Ingredients / Products Feeding Directions snip... _animal protein_

 

http://www.surefed.com/deer.htm

 

snip...

 

DEPARTMENT OF HEALTH & HUMAN SERVICES

 

PUBLIC HEALTH SERVICEFOOD AND DRUG ADMINISTRATION

 

April 9, 2001

 

WARNING LETTER

 

01-PHI-12CERTIFIED MAILRETURN RECEIPT REQUESTED

 

Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy Lake, PA 16145 PHILADELPHIA DISTRICT Tel: 215-597-4390

 

Dear Mr. Raymond:

 

Food and Drug Administration Investigator Gregory E. Beichner conducted an inspection of your animal feed manufacturing operation, located in Sandy Lake, Pennsylvania, on March 23,2001, and determined that your firm manufactures animal feeds including feeds containing prohibited materials.

 

The inspection found significant deviations from the requirements set forth in Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being manufactured at this facility to be misbranded within the meaning of Section 403(f), of the Federal Food, Drug, and Cosmetic Act (the Act).

 

Our investigation found failure to label your swine feed with the required cautionary statement "Do Not Feed to cattle or other Ruminants" The FDA suggests that the statement be distinguished by different type-size or color or other means of highlighting the statement so that it is easily noticed by a purchaser.

 

In addition, we note that you are using approximately 140 pounds of cracked corn to flush your mixer used in the manufacture of animal feeds containing prohibited material. This flushed material is fed to wild game including deer, a ruminant animal.

 

Feed material which may potentially contain prohibited material should not be fed to ruminant animals which may become part of the food chain.

 

The above is not intended to be an all-inclusive list of deviations from the regulations. As a manufacturer of materials intended for animal feed use, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance with the law.

 

We have enclosed a copy of FDA's Small Entity Compliance Guideto assist you with complying with the regulation...

 

http://www.fda.gov/foi/warning_letters/g1115d.pdf

 

 snip...end...full text ;

 

2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed EMC 1 Terry S. Singeltary Sr. Vol #: 1

 

http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm

 

http://www.fda.gov/ohrms/dockets/dailys/01/Oct01/101501/101501.htm

 

see my full text submission here ;

 

http://madcowfeed.blogspot.com/2008/07/docket-03d-0186-fda-issues-draft.html

 

http://chronic-wasting-disease.blogspot.com/2016/03/docket-no-fda-2003-d-0432-formerly-03d.html

 

http://www.fda.gov/downloads/AnimalVeterinary/GuidanceComplianceEnforcement/GuidanceforIndustry/ucm052506.pdf

 

 

>>> We can prevent, effectively treat, and make an Alzheimer’s cure possible by 2025.

 

PREVENT

 

this must be on the forefront of research i.e. ‘iatrogenic’ transmission.

 

Alzheimer’s disease, iatrogenic, and Transmissible Spongiform Encephalopathy TSE Prion disease, that is the question ???

 

>>> The only tenable public line will be that "more research is required’’ <<<

 

>>> possibility on a transmissible prion remains open<<<

 

O.K., so it’s about 23 years later, so somebody please tell me, when is "more research is required’’ enough time for evaluation ?

 

http://collections.europarchive.org/tna/20080102232842/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf

 

http://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf

 

SWISS MEDICAL WEEKLY

 

Alzheimer-type brain pathology may be transmitted by grafts of dura mater 26/01/2016 Singeltary comment ;

 

http://blog.smw.ch/alzheimer-type-brain-pathology-may-be-transmitted-by-grafts-of-dura-mater/#comment-89016

 

re-Evidence for human transmission of amyloid-β pathology and cerebral amyloid angiopathy

 

Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26 April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated online 11 September 2015 Erratum (October, 2015)

 

snip...see full Singeltary Nature comment here;

 

http://www.nature.com/nature/journal/v525/n7568/full/nature15369.html#/comments

 

Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease

 

*** Singeltary comment PLoS ***

 

Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?

 

Posted by flounder on 05 Nov 2014 at 21:27 GMT

 

http://www.plosone.org/annotation/listThread.action?root=82860

 

Sunday, November 22, 2015

 

*** Effect of heating on the stability of amyloid A (AA) fibrils and the intra- and cross-species transmission of AA amyloidosis Abstract

 

Amyloid A (AA) amyloidosis is a protein misfolding disease characterized by extracellular deposition of AA fibrils. AA fibrils are found in several tissues from food animals with AA amyloidosis. For hygienic purposes, heating is widely used to inactivate microbes in food, but it is uncertain whether heating is sufficient to inactivate AA fibrils and prevent intra- or cross-species transmission. We examined the effect of heating (at 60 °C or 100 °C) and autoclaving (at 121 °C or 135 °C) on murine and bovine AA fibrils using Western blot analysis, transmission electron microscopy (TEM), and mouse model transmission experiments. TEM revealed that a mixture of AA fibrils and amorphous aggregates appeared after heating at 100 °C, whereas autoclaving at 135 °C produced large amorphous aggregates. AA fibrils retained antigen specificity in Western blot analysis when heated at 100 °C or autoclaved at 121 °C, but not when autoclaved at 135 °C. Transmissible pathogenicity of murine and bovine AA fibrils subjected to heating (at 60 °C or 100 °C) was significantly stimulated and resulted in amyloid deposition in mice. Autoclaving of murine AA fibrils at 121 °C or 135 °C significantly decreased amyloid deposition. Moreover, amyloid deposition in mice injected with murine AA fibrils was more severe than that in mice injected with bovine AA fibrils. Bovine AA fibrils autoclaved at 121 °C or 135 °C did not induce amyloid deposition in mice. These results suggest that AA fibrils are relatively heat stable and that similar to prions, autoclaving at 135 °C is required to destroy the pathogenicity of AA fibrils. These findings may contribute to the prevention of AA fibril transmission through food materials to different animals and especially to humans.

 

Purchase options Price * Issue Purchase USD 511.00 Article Purchase USD 54.00

 

http://www.tandfonline.com/doi/abs/10.3109/13506129.2015.1095735?journalCode=iamy20

 

http://betaamyloidcjd.blogspot.com/2015/11/effect-of-heating-on-stability-of.html

 

*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***

 

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

 

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

 

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract

 

CONFIDENTIAL

 

Transmissible Spongiform Encephalopathy TSE Prion and how Politics and Greed by the Industry spread madcow type diseases from species to species and around the globe

 

TSE PRIONS AKA MAD COW TYPE DISEASE, LIONS AND TIGERS AND BEARS, OH MY!

 

http://transmissiblespongiformencephalopathy.blogspot.com/2016/08/transmissible-spongiform-encephalopathy.html

 

http://transmissiblespongiformencephalopathy.blogspot.com/

 

Monday, August 22, 2016

 

CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES

 

http://creutzfeldt-jakob-disease.blogspot.com/2016/08/creutzfeldt-jakob-disease-usa-2015.html

 

*** Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle ***

 

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

 

snip...

 

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/mb/m09/tab05.pdf

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/mb/m09a/tab01.pdf

 

http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1987/06/10004001.pdf

 

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

 

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...

 

http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

 

”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” ...page 26.

 

http://collections.europarchive.org/tna/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

 

Terry S. Singeltary, Sr Bacliff, Tex

 

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

 

http://jama.jamanetwork.com/article.aspx?articleid=1031186

 

IBNC Tauopathy or TSE Prion disease, it appears, no one is sure

 

Posted by flounder on 03 Jul 2015 at 16:53 GMT

 

http://www.plosone.org/annotation/listThread.action?root=86610

 

Human Prion Diseases in the United States Robert C. Holman ,

 

Ermias D. Belay, Krista Y. Christensen, Ryan A. Maddox, Arialdi M. Minino, Arianne M. Folkema, Dana L. Haberling, Teresa A. Hammett, Kenneth D. Kochanek, James J. Sejvar, Lawrence B. Schonberger

 

PLOS

 

Published: January 1, 2010 • http://dx.doi.org/10.1371/journal.pone.0008521

 

re-Human Prion Diseases in the United States

 

Posted by flounder on 01 Jan 2010 at 18:11 GMT

 

I kindly disagree with your synopsis for the following reasons ;

 

The Pathological Protein:

 

Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases

 

Philip Yam

 

*** ''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population'' ***

 

http://www.amazon.com/The-Pathological-Protein-Chronic-Diseases/dp/0387955089

 

http://transmissiblespongiformencephalopathy.blogspot.com/2014/12/terry-s-singeltary-sr-publications-tse.html

 

The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

 

Tracking spongiform encephalopathies in North America

 

Original

 

Xavier Bosch

 

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt—Jakob ...

 

http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/abstract

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

 

Terry S. Singeltary, Sr Bacliff, Tex

 

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

 

http://jama.jamanetwork.com/article.aspx?articleid=1031186

 

26 March 2003

 

Terry S. Singeltary, retired (medically) CJD WATCH

 

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

 

http://www.neurology.org/content/60/2/176/reply#neurology_el_535

 

Sent: Monday, January 08,2001 3:03 PM

 

TO: freas@CBS5055530.CBER.FDA.GOV

 

FDA CJD BSE TSE Prion Scientific Advisors and Consultants Staff January 2001 Meeting Singeltary Submission

 

2001 FDA CJD TSE Prion Singeltary Submission

 

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

 

2 January 2000

 

British Medical Journal

 

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

 

http://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well

 

15 November 1999

 

British Medical Journal

 

vCJD in the USA * BSE in U.S.

 

http://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us

 

2001 FDA CJD TSE Prion Singeltary Submission TSEAC

 

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf

 

Terry S. Singeltary Sr. Bacliff, Texas USA 77518 flounder9@verizon.net

 

Thursday, September 29, 2016

 

A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia

 

http://vcjd.blogspot.com/2016/09/a-case-cluster-of-variant-creutzfeldt.html

 

 

======================END...TSS==================

 

 

Terry S. Singeltary Sr., Bacliff, Texas USA 77518 flounder9@verizon.net