The PrP gene polymorphisms at codons 142 (I/M), 154 (R/H), 211 (R/Q), 222 (Q/K) and 240 (S/P) and their association with susceptibility to classical scrapie infection were investigated in five French goat herds displaying a high disease prevalence (>10%). On the basis of PrP(Sc) detection in the central nervous system and in various lymphoid tissues, 301 of 1343 goats were found to be scrapie i...

The pathogenesis of natural scrapie and other prion diseases remains unclear. Examining transcriptome variations in infected versus control animals may highlight new genes potentially involved in some of the molecular mechanisms of prion-induced pathology. The aim of this work was to identify disease-associated alterations in the gene expression profiles of the caudal medulla oblongata (MO) in ...

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...

Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk are contagious prion diseases where environmental reservoirs are directly implicated in the transmission of disease. In this study the effectiveness of recommended scrapie farm decontamination regimes was evaluated by a sheep bioassay using buildings naturally contaminated with scrapie. Pens within a farm building were treated...

Previously, Multimer Detection System (MDS) detected scrapie infected lambs of 8 mo age at pre-clinical stage in comparison with the normal controls. Above lamb were born from scrapie infected parent sheep (VRQ/VRQ). Here, MDS was challenged twice blindly with scrapie sheep blood samples from pre-clinical stages. These sheep showed no symptoms and they died of scrapie after 24 mo or more. Both ...

Scrapie is a transmissible neurodegenerative disease of sheep and goats. The neuropathological changes include vacuolation, astrocytosis, the development of amyloid plaques in some instances, and neuronal loss. The mechanisms involved in neuronal cell death in scrapie are not known. Recently, we reported the presence of oxidative stress in the brains of scrapie-infected animals and suggested th...

Atypical/Nor98 scrapie was first identified in 1998 in Norway. It is now considered as a worldwide disease of small ruminants and currently represents a significant part of the detected transmissible spongiform encephalopathies (TSE) cases in Europe. Atypical/Nor98 scrapie cases were reported in ARR/ARR sheep, which are highly resistant to BSE and other small ruminants TSE agents. The biology a...

Mice devoid of PrP are resistant to scrapie and fail to replicate the agent. Introduction of transgenes expressing PrP into such mice restores susceptibility to scrapie. We find that truncated PrP devoid of the five copper binding octarepeats still sustains scrapie infection; however, incubation times are longer and prion titers and protease-resistant PrP are about 30-fold lower than in wild-ty...

Ž . We investigated the distribution of EcoRI restriction fragment length polymorphism RFLP and tested EcoRI and HindIII RFLPs to ascertain the occurrence of scrapie in Suffolk sheep. Genomic DNA samples were collected from 527 Ž . animals and typed for EcoRI RFLP by Southern blot analysis using the PrP gene clone probe pNPU42 . The average e1 frequency in Suffolk sheep was 64.4% and ranged fro...

BACKGROUND Classical scrapie is a transmissible spongiform encephalopathy (TSE) that affects sheep and goats. Our previous bioassay studies in lambs revealed that scrapie prions could be detected in association with peripheral blood monocular cells (PBMC), B lymphocytes and platelet-rich plasma fractions. In the present study, bioassay in lambs was again used to determine if scrapie prions are ...