Diversity in Neuroanatomical Distribution of Abnormal Prion Protein in Atypical Scrapie

نویسندگان

  • Alice Nentwig
  • Anna Oevermann
  • Dagmar Heim
  • Catherine Botteron
  • Karola Zellweger
  • Cord Drögemüller
  • Andreas Zurbriggen
  • Torsten Seuberlich
چکیده

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.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Atypical Scrapie Isolates Involve a Uniform Prion Species with a Complex Molecular Signature

The pathobiology of atypical scrapie, a prion disease affecting sheep and goats, is still poorly understood. In a previous study, we demonstrated that atypical scrapie affecting small ruminants in Switzerland differs in the neuroanatomical distribution of the pathological prion protein (PrP(d)). To investigate whether these differences depend on host-related vs. pathogen-related factors, we tra...

متن کامل

Immunological characterization of abnormal prion protein from atypical scrapie cases in sheep using a panel of monoclonal antibodies.

After the implementation of an active surveillance programme for scrapie in sheep in the EU, the number of diagnosed classical scrapie cases rose sharply and a novel kind of so-called atypical scrapie case was discovered. These atypical scrapie cases display unusual features concerning the distribution of the abnormal prion protein (PrP(Sc)) in the brain, a distinct electrophoretic profile of P...

متن کامل

Classical and Atypical Scrapie in Sheep and Goats

W.-Q. Zou and P. Gambetti (eds.), Prions and Diseases: Volume 2, Animals, Humans and the Environment, DOI 10.1007/978-1-4614-5338-3_2, © Springer Science+Business Media New York 2013 Abstract Scrapie is a naturally occuring transmissible spongiform encephalopathy (TSE) in sheep, goat and muf fl ons almost world-wide and is known for about 250 years. It is characterized by the accumulation of an...

متن کامل

Polymorphism of Prion Protein Gene (PRNP) in Iranian Holstein and Two Local Cattle Populations (Golpayegani and Sistani) of Iran

Bovine spongiform encephalopathy (BSE) is a fatal infectious neurodegenerative disease in cattle, characterized by the accumulation of an abnormal, proteaseresistant prion protein (PrPSc) in the brain. BSE is similar to scrapie in sheep and goats and Creuzfeldt-Jakob disease in humans. Susceptibility in cattle hasbeen shown to be under the influence of two polymorphic locations, which are...

متن کامل

Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice.

Twenty-four atypical scrapie cases from sheep with different prion protein genotypes from Great Britain were transmitted to transgenic tg338 and/or TgshpXI mice expressing sheep PrP alleles, but failed to transmit to wild-type mice. Mean incubation periods were 200-300 days in tg338 mice and 300-500 days in TgshpXI mice. Survival times in C57BL/6 and VM/Dk mice were >700 days. Western blot anal...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • PLoS Pathogens

دوره 3  شماره 

صفحات  -

تاریخ انتشار 2007