title: Diversity in neuroanatomical distribution of abnormal prion protein in atypical scrapie.
abstract: Scrapie is a transmissible spongiform encephalopathy (TSE) in sheep and goats. In recent years, atypical scrapie cases were identified that differed from classical scrapie in the molecular characteristics of the disease-associated pathological prion protein (PrP(sc)). In this study, we analyze the molecular and neuropathological phenotype of nine Swiss TSE cases in sheep and goats. One sheep was identified as classical scrapie, whereas six sheep, as well as two goats, were classified as atypical scrapie. The latter revealed a uniform electrophoretic mobility pattern of the proteinase K-resistant core fragment of PrP(sc) distinct from classical scrapie regardless of the genotype, the species, and the neuroanatomical structure. Remarkably different types of neuroanatomical PrP(sc) distribution were observed in atypical scrapie cases by both western immunoblotting and immunohistochemistry. Our findings indicate that the biodiversity in atypical scrapie is larger than expected and thus impacts on current sampling and testing strategies in small ruminant TSE surveillance.
authors.affiliation: NeuroCenter, Reference Laboratory for TSE in Animals, Vetsuisse Faculty, University of Berne, Berne, Switzerland.
authors.forename: Alice
authors.initials: A
authors.lastname: Nentwig
authors.forename: Anna
authors.initials: A
authors.lastname: Oevermann
authors.forename: Dagmar
authors.initials: D
authors.lastname: Heim
authors.forename: Catherine
authors.initials: C
authors.lastname: Botteron
authors.forename: Karola
authors.initials: K
authors.lastname: Zellweger
authors.forename: Cord
authors.initials: C
authors.lastname: Drögemüller
authors.forename: Andreas
authors.initials: A
authors.lastname: Zurbriggen
authors.forename: Torsten
authors.initials: T
authors.lastname: Seuberlich
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D014644:Genetic Variation; D015511:Goat Diseases / Q000378:metabolism* / Q000473:pathology; D006041:Goats; D007124:Immunoenzyme Techniques; D008969:Molecular Sequence Data; D018620:PrPSc Proteins / Q000235:genetics / Q000378:metabolism*; D012608:Scrapie / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D012756:Sheep
chemical_list: D018620:PrPSc Proteins