Diversity in Neuroanatomical Distribution of Abnormal Prion Protein in Atypical Scrapie 英文参考文献.docVIP

DiversityinNeuroanatomicalDistribution ofAbnormalPrionProtein inAtypicalScrapie Alice Nentwig1,Anna Oevermann1,Dagmar Heim2,Catherine Botteron1,Karola Zellweger1,Cord Dro¨gemu¨ller3, Andreas Zurbriggen1,Torsten Seuberlich1* 1 NeuroCenter, Reference Laboratory for TSE in Animals, Vetsuisse Faculty, University of Berne, Berne, Switzerland, 2 Federal Veterinary Office, Liebefeld, Switzerland, 3InstituteofGenetics,VetsuisseFaculty,UniversityofBerne,Berne,Switzerland Scrapieisatransmissiblespongiformencephalopathy(TSE)insheepandgoats.Inrecentyears,atypicalscrapiecases were identified that differed from classical scrapie in the molecular characteristics of the disease-associated pathologicalprionprotein(PrPsc).Inthisstudy,weanalyzethemolecularandneuropathologicalphenotypeofnine SwissTSEcasesinsheepandgoats.Onesheepwasidentifiedasclassicalscrapie,whereassixsheep,aswellastwo goats, were classified as atypical scrapie. The latter revealed a uniform electrophoretic mobility pattern of the proteinaseK–resistantcorefragmentofPrPsc distinctfromclassicalscrapieregardlessofthegenotype,thespecies, andtheneuroanatomicalstructure.RemarkablydifferenttypesofneuroanatomicalPrPscdistributionwereobservedin atypical scrapie cases by both western immunoblotting and immunohistochemistry. Our findings indicate that the biodiversityinatypicalscrapieislargerthanexpectedandthusimpactsoncurrentsamplingandtestingstrategiesin smallruminantTSEsurveillance. Citation:NentwigA,OevermannA,HeimD,BotteronC,ZellwegerK,etal.(2007)Diversityinneuroanatomicaldistributionofabnormalprionproteininatypicalscrapie.PLoS Pathog3(6):e82.doi:10.1371/journal.ppat.0030082 by molecular PrPres typing [8]: classical scrapie, atypical scrapie,andBSE. Introduction Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases that are caused by prions [1] and include, among others, Creutzfeldt-Jakob and Gerstmann-Stra¨ussler-Scheinker disease (GSS) in humans, bovine spongiform encephalopat