SCIENTIFIC REPORT OF EFSA 
Scientific and technical assistance on the provisional results of the study 
on genetic resistance to Classical scrapie in goats in Cyprus1 
European Food Safety Authority2, 3 
European Food Safety Authority (EFSA), Parma, Italy 
ABSTRACT 
This EFSA Scientific Report reviews and discusses the provisional results 
of a study (EURL/Cypriot study) on genetic resistance to Classical scrapie in 
goats in Cyprus. It is concluded that the provisional results obtained in the 
study further support the lower susceptibility to Classical scrapie in goats 
carrying the D146 and S146 alleles compared to wild type (N146N) goats. The 
results from intracerebral challenge are not compatible with a level of 
resistance as high as the one observed in sheep carrying the ARR allele or in 
goats carrying the K222 allele. Final results from the oral challenge will be 
crucial in determining the level of resistance associated with the D146 and S146 
alleles. Furthermore, it is concluded that the provisional results obtained in 
the study are compatible with the possibility to use the D146 and S146 alleles 
to build a genetic strategy to control and eradicate Classical scrapie in goats 
in Cyprus. However, the success of such a strategy will be determined by the 
level of resistance associated with the D146 and S146 alleles against infection 
with all the different TSE agents proved to be circulating in Cyprus, which at 
this stage of the EURL/Cypriot study remains to be definitively assessed. In 
addition, as compared to the results of the model developed in the study, it is 
concluded that the efficiency of the implementation in the field of a breeding 
strategy selecting for the D146 and S146 alleles may be lower due to potential 
practical constraints related to the management of genetic diversity, to the 
selection for production and health traits and to the need of moving animals for 
breeding purposes in Cyprus. Recommendations on aspects that may be considered 
when completing the study are formulated. 
© European Food Safety Authority, 2012 
KEY WORDS 
TSE, Classical scrapie, goat, genetic resistance, breeding programme, 
Cyprus. 
 snip... 
 Interim Conclusions 
The oral challenge studies, the management cull and the whole herd cull 
data all support the previously published field observations that on one side 
the genotype N146N is particularly associated with scrapie susceptibility and on 
the other side the genotypes N146D, N146S, D146D, D146S and S146S are associated 
with a degree of resistance. 
All genotypes can succumb to challenge by the intracerebral route, but the 
resulting phenotype is different when comparing the animals carrying genotype 
N146N with all the others (most notably, no detection of PrPSc in the periphery 
of infected animals with all the non-N146N-genotypes). 
It is important that the oral challenges are continued to endpoint to 
establish the relative resistance of other genotypes to challenge by this more 
natural route Appendix A to Scientific Report of EFSA, EFSA Journal 
2012;10(11):2972 
Cyprus/EURL resistance in goats protocol Report May 2012 
Page 13 of 13 
All components of the study reinforce previously published UK caprine data3 
which indicates that the current ELISA rapid test screen has considerably lower 
sensitivity (approx 50%) than immunohistochemistry. 
The wider issue of discriminatory testing for BSE vs scrapie may need to be 
reviewed (regardless of genotype), since data from these studies suggest that 
direct extrapolation from ovine data may not be appropriate for all caprine 
isolates. 
The TSE European Union Reference Laboratory at the Animal Health and 
Veterinary Laboratory Agency New Haw, UK 
The Government Veterinary Services, Cyprus 
May 2012 
Wednesday, January 18, 2012
BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE 
February 1, 2012 
posted January 18, 2012 
BSE in goats can be mistaken for scrapie 
Bovine spongiform encephalopathy in goats could be misdiagnosed as scrapie 
in the absence of appropriate discriminatory tests, and such misidentification 
occurred at least once before such tests were developed, according to a report 
released in December. 
The article, "Isolation of prion with BSE properties from farmed goat" 
(Emerging Infectious Diseases 2011;17:2253-2261), indicates BSE can affect small 
ruminants under natural conditions and that the condition can be misdiagnosed. 
The agent that causes scrapie is not known to infect humans, but consumption of 
beef contaminated with the prions that cause BSE is connected with variant 
Creutzfeldt-Jakob disease, a neurodegenerative disorder in humans. 
The report calls for continued extensive surveillance and breeding plans to 
prevent BSE outbreaks among small ruminants. Such outbreaks could harm public 
health. 
The authors stated in the text that the misdiagnosis occurred in 1990 in 
the United Kingdom. The case had been identified as suspected BSE in 2006 
because differential immunohistochemical analysis of fixed brain tissue produced 
a signature indistinguishable from BSE. The authors of the recent report used a 
bioassay to confirm the BSE diagnosis. 
The sample collected in 1990 was among 26 historic samples collected from 
1984-2002, the report states. 
The report indicates the U.K. goat and a goat in France found to have BSE 
in 2005 both likely became infected through contaminated food supplements. 
While BSE lesions are contained mainly within nervous tissue in cattle, the 
report states "in small ruminants the BSE agent is widely distributed in 
peripheral tissues and can be transmitted horizontally." Feed ban measures alone 
would be insufficient for controlling a BSE outbreak in small ruminants, 
according to the report. 
"Also, it would be impossible to prevent BSE from entering the human food 
chain through consumption of food products derived from small ruminants," the 
report states. 
Discussion 
We confirmed that the agent responsible for TSE in a UK goat, which was 
initially reported as scrapie in 1990 and subsequently as suspected BSE in 2006 
(16), was a BSE agent. This conclusion was based on bioassay of nervous tissue 
in mice demonstrating similarities of histopathologic lesions, PrPSc mapping in 
the brain, and WB of PrPSc with those of mice inoculated with BSE from various 
ovine, caprine, and bovine sources. 
Saturday, December 3, 2011 
Isolation of Prion with BSE Properties from Farmed Goat Volume 17, Number 
12—December 2011 
snip...see full text ; 
Scientific Opinion on genetic TSE resistance in goats in all European Union 
Member States Question number: EFSA-Q-2009-00448
Adopted: 21 October 2009 Summary (0.1Mb)
Opinion (0.3Mb)
Summary
Following a request from the European Commission, the Panel on Biological 
Hazards (BIOHAZ) was asked to deliver a scientific opinion on genetic resistance 
to Transmissible Spongiform Encephalopathies (TSE) in goats.
For a first part of that request, the BIOHAZ Panel adopted on 5th March 
2009 a scientific opinion on the scientific validity of a study carried out by 
the Cypriot authorities under the auspices of the Community Reference Laboratory 
(CRL) for TSEs. That scientific opinion also indicated to what extent the 
information contained in the study could be used as relevant tools to control 
Classical scrapie in Cyprus.
In the current scientific opinion the BIOHAZ Panel addresses genetic 
resistance as a relevant tool for breeding for resistance to all TSEs of goats 
(including Atypical scrapie and BSE) in all the Member States (MSs) (except for 
Classical scrapie in Cyprus).
To carry out this task, available scientific knowledge on genetic TSE 
resistance in goats in the EU is reviewed, addressing those PRNP polymorphisms 
for which a capacity to provide resistance to TSEs in goats has been (or is 
being) investigated. Details tailored to the different TSEs found in this small 
ruminants (i.e. Classical scrapie, Atypical scrapie and BSE) are also considered 
and presented.
Further on, the feasibility of a large-scale breeding program in animal 
populations would need to be supported by a sound logistical and technical 
infrastructure in any given territory. In order to collect preliminary data that 
could help to evaluate the specific situation in the different EU MSs, a 
questionnaire was developed and circulated among the EFSA BSE-TSE Network. The 
results of the analysis of the replies received are also presented 
herewith.
The BIOHAZ Panel concluded that there are encouraging but as yet incomplete 
data to consider supporting a breeding programme for resistance in goats against 
Classical scrapie in all EU MSs, and ongoing studies are expected to provide a 
more robust scientific background in the coming years. On the other hand, at 
this moment there are not enough data available to consider supporting a 
breeding programme for resistance against Atypical scrapie and BSE in goats in 
all EU MSs. Experiments are ongoing on BSE in goats and results will be 
available in the next years. Furthermore, there are limited data suggesting that 
an allele (H154) might confer resistance to Classical scrapie but increase 
susceptibility to Atypical scrapie.
The frequency of the wild type allele, which is known to confer 
susceptibility to Classical scrapie, is high in all goat breeds considered. 
Thus, selection for putative resistance alleles will be slow, complicated and 
highly dependent on breeding structure.
It is acknowledged that any large scale breeding programme for TSE 
resistance in goats must take into consideration key elements related to the 
current dissemination of potentially TSE protective polymorphisms in the goat 
population of each EU MS and the characterisation of the real protection 
provided by those polymorphisms. At present, only a few EU MSs seem to have in 
place the necessary elements to introduce a breeding for resistance programme 
for Classical scrapie in goats.
The BIOHAZ Panel makes a series of recommendations on new investigations in 
order to assess the efficacy of breeding for the candidate PRNP alleles as a 
mean to control TSEs in goats. Furthermore, research on the possible adverse 
effects of the candidate PRNP polymorphisms on other production traits should be 
encouraged. In addition, it is recommended that a breeding for resistance 
programme for TSE in goats is first implemented in the seven EU MSs with the 
largest goat population as this would have the most impact.
Published: 9 November 2009
OPINION
Tuesday, November 10, 2009
A retrospective immunohistochemical study reveals atypical scrapie has 
existed in the United Kingdom since at least 1987
Brief Research Reports
-------- Original Message -------- 
Subject: Twelve Greek goats were found to be suffering from the 
brain-wasting disease scrapie in the first half of 2004 
Date: Wed, 19 Jan 2005 13:30:26 –0600 
From: "Terry S. Singeltary Sr." 
To: Bovine Spongiform Encephalopathy 
CC: cjdvoice@yahoogroups.com 
Wednesday January 19, 2005 
Brain disease in Greek goats 
Twelve Greek goats were found to be suffering from the brain-wasting 
disease scrapie in the first half of 2004, EU figures made public yesterday 
reveal. 
The data, issued by the European Food Safety Authority (EFSA), show that 12 
cases of transmissible spongiform encephalopathies (TSE) were discovered in 
Greece, eight in Cyprus and 26 in France out of some 17,294 goats tested 
throughout the EU in 2004. The figures were made public by Left Coalition 
Synaspismos MEP Dimitris Papadopoulos. 
Some 100 Europeans have died from the human form of bovine spongiform 
encephalopathy (BSE), or  mad cow disease,  a strain of the TSE group. 
Meanwhile, tests are continuing in the case of a French goat slaughtered in 
2002, which experts think may have developed BSE. The EU bans the use of milk 
and meat from herds affected by a TSE case. 
TSS 
-------- Original Message -------- 
Subject: Twelve Greek goats were found to be suffering from the 
brain-wasting disease scrapie in the first half of 2004 
Date: Wed, 19 Jan 2005 13:30:26 –0600 
From: "Terry S. Singeltary Sr." 
To: Bovine Spongiform Encephalopathy 
CC: cjdvoice@yahoogroups.com 
Wednesday January 19, 2005 
Brain disease in Greek goats 
Twelve Greek goats were found to be suffering from the brain-wasting 
disease scrapie in the first half of 2004, EU figures made public yesterday 
reveal. 
The data, issued by the European Food Safety Authority (EFSA), show that 12 
cases of transmissible spongiform encephalopathies (TSE) were discovered in 
Greece, eight in Cyprus and 26 in France out of some 17,294 goats tested 
throughout the EU in 2004. The figures were made public by Left Coalition 
Synaspismos MEP Dimitris Papadopoulos. 
Some 100 Europeans have died from the human form of bovine spongiform 
encephalopathy (BSE), or  mad cow disease,  a strain of the TSE group. 
Meanwhile, tests are continuing in the case of a French goat slaughtered in 
2002, which experts think may have developed BSE. The EU bans the use of milk 
and meat from herds affected by a TSE case. 
TSS 
Comment from Terry S Singeltary, CJD WATCH/VOICE
| Document ID: APHIS-2007-0033-0002 | Document Type: Public Submission | 
| 
This is comment on 
Proposed Rule: Agricultural Bioterrorism Protection Act of 2002; 
Biennial Review and Republication of the Select Agent and Toxin 
ListDocket ID: | RIN:0579-AC53 | 
Topics: No Topics associated with this 
document
View 
Document:
snip...
Under APHIS-PPQ’s agriculture quarantine inspection monitoring, 584 air 
passengers from Greece were sampled for items of agricultural interest in fiscal 
year 2000. Of these passengers, 14 carried meat (non-pork) items that could 
potentially transmit pathogens that cause BSE; most passengers carried from one 
to two kilograms (kg) of meat, although one passenger in November 1999 carried 
23 kg of meat in a suitcase. Florida, Massachusetts, and New York were the 
reported destinations of these passengers. None of the passengers with meat 
items reported plans to visit or work on a ranch or farm while in the US.
Source: US Department of Transportation, and APHIS-PPQ Agricultural 
Quarantine Inspection data base

 
No comments:
Post a Comment
Note: Only a member of this blog may post a comment.