Document Detail


Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease.
MedLine Citation:
PMID:  16400018     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Molecular typing of the abnormal form of the prion protein (PrP(Sc)) has come to be regarded as a powerful tool in the investigation of the prion diseases. All evidence thus far presented indicates a single PrP(Sc) molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). Here we show for the first time that the PrP(Sc) that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type 1 component. This minority type 1 PrP(Sc) was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective of brain region examined, and was also present in the variant Creutzfeldt-Jakob disease tonsil. The quantitative balance between PrP(Sc) types was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type mice and was also found in bovine spongiform encephalopathy cattle brain, indicating that the agent rather than the host specifies their relative representation. These results indicate that PrP(Sc) molecular typing is based on quantitative rather than qualitative phenomena and point to a complex relationship between prion protein biochemistry, disease phenotype and agent strain.
Authors:
Helen M Yull; Diane L Ritchie; Jan P M Langeveld; Fred G van Zijderveld; Moira E Bruce; James W Ironside; Mark W Head
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of pathology     Volume:  168     ISSN:  0002-9440     ISO Abbreviation:  Am. J. Pathol.     Publication Date:  2006 Jan 
Date Detail:
Created Date:  2006-01-09     Completed Date:  2006-03-23     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  0370502     Medline TA:  Am J Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  151-7     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Sequence
Animals
Antibodies, Monoclonal
Blotting, Western
Brain Chemistry*
Cattle
Creutzfeldt-Jakob Syndrome / metabolism*
Encephalopathy, Bovine Spongiform / metabolism
Humans
Immunohistochemistry
Mice
Molecular Sequence Data
PrPSc Proteins / chemistry*,  genetics
Grant Support
ID/Acronym/Agency:
G0600953//Medical Research Council; G0900580//Medical Research Council
Chemical
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/PrPSc Proteins
Comments/Corrections

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