Protein aggregate spreading in neurodegenerative diseases: Problems and perspectives

Protein aggregate spreading in neurodegenerative diseases: Problems and perspectives


doi:10.1016/j.neures.2011.05.008 | How to Cite or Link Using DOI Permissions & Reprints

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Seung-Jae Leea, c, , , Hee-Sun Lima, c, Eliezer Masliahd and He-Jin Leeb, c

a Department of Biomedical Science and Technology, Konkuk University, Seoul 143-701, Republic of Korea

b Department of Anatomy School of Medicine, Konkuk University, Seoul 143-701, Republic of Korea

c Institute of Biomedical Science and Technology, Konkuk University, Seoul 143-701, Republic of Korea

d Department of Neurosciences and Pathology, School of Medicine, University of California San Diego, La Jolla, CA 92093-0624, USA

Received 4 March 2011; revised 13 May 2011; accepted 16 May 2011. Available online 20 May 2011.


Abstract


Progressive accumulation of specific protein aggregates is a defining feature of many major neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, fronto-temporal dementia, Huntington's disease, and Creutzfeldt–Jakob disease (CJD). Findings from several recent studies have suggested that aggregation-prone proteins, such as tau, a-synuclein, polyglutamine-containing proteins, and amyloid-ß, can spread to other cells and brain regions, a phenomenon considered unique to prion disorders, such as CJD and bovine spongiform encephalopathy. Cell-to-cell propagation of protein aggregates may be the general underlying principle for progressive deterioration of neurodegenerative diseases. This may also have significant implications in cell replacement therapies, as evidenced by the propagation of a-synuclein aggregates from host to grafted cells in long-term transplants in Parkinson's patients. Here, we review recent progress in protein aggregate propagation in experimental model systems and discuss outstanding questions and future perspectives. Understanding the mechanisms of this pathological spreading may open the way to unique opportunities for development of diagnostic techniques and novel therapies for protein misfolding-associated neurodegenerative diseases.


Keywords: Protein aggregation; Alzheimer's disease; Parkinson's disease; Huntington's disease; Prion


Abbreviations: CJD, Creutzfeldt–Jakob disease; AD, Alzheimer's disease; PD, Parkinson's disease; HD, Huntington's disease; Aß, amyloid-ß; polyQ, polyglutamine; CNS, central nervous system; NFTs, neurofibrillary tangles; NTs, neuropil threads; APP, amyloid precursor protein


http://www.sciencedirect.com/science/article/pii/S0168010211001325




Saturday, January 22, 2011

Alzheimer's, Prion, and Neurological disease, and the misdiagnosis there of, a review 2011

http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/alzheimers-prion-and-neurological.html




Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html




Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

http://creutzfeldt-jakob-disease.blogspot.com/2011/04/sporadic-cjd-rising-text-and-figures-of.html




PLEASE REMEMBER ;

The Akron, Ohio-based CJD Foundation said the Center for Disease Control revised that number in October of 2004 to about one in 9,000 CJD cases per year in the population group age 55 and older.

HAVE YOU GOT YOUR CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO ROUTE AND SOURCE OF THE TSE AGENT THAT KILLED YOUR LOVED ONE ???

Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html



http://cjdquestionnaire.blogspot.com/






USA PRION FUNDING


"which includes the ___elimination___ of Prion activities ($5,473,000),"


All Other Emerging and Zoonotic Infectious Diseases CDC's FY 2012 request of $52,658,000 for all other emerging and zoonotic infectious disease activities is a decrease of $13,607,000 below the FY 2010 level, which includes the elimination of Prion activities ($5,473,000), a reduction for other cross-cutting infectious disease activities, and administrative savings. These funds support a range of critical emerging and zoonotic infectious disease programs such Lyme Disease, Chronic Fatigue Syndrome, and Special Pathogens, as well as other activities described below.

http://www.cdc.gov/fmo/topic/Budget%20Information/appropriations_budget_form_pdf/FY2012_CDC_CJ_Final.pdf






Sunday, June 5, 2011


PRION TSE FUNDING, WHAT ARE THE PRIORITIES of APHCA, ASEAN, OIE, Korea, and USA ?


http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/prion-tse-funding-what-are-priorities.html







Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518