Scrapie is a fatal neurodegenerative disease of sheep and goats belonging to the group of transmissible spongiform encephalopathies (TSE). TSEs are characterised by distinctive pathology, the involvement of a hostencoded prion protein (PrP) in disease development and genetic control of susceptibility. Polymorphisms in three different codons of the ovine prion protein (PrP) gene (136, 154, 171) ...

Natural scrapie transmission from infected ewes to their lambs is thought to occur by the oral route around the time of birth. However the hypothesis that scrapie transmission can also occur before birth (in utero) is not currently favoured by most researchers. As scrapie is an opportunistic infection with multiple infection routes likely to be functional in sheep, definitive evidence for or ag...

BACKGROUND Diagnosis based on prion detection in lymph nodes of sheep and goats can improve active surveillance for scrapie and, if it were circulating, for bovine spongiform encephalopathy (BSE). With sizes that allow repetitive testing and a location that is easily accessible at slaughter, retropharyngeal lymph nodes (RLN) are considered suitable organs for testing. Western blotting (WB) of b...

Introduction Scrapie is a fatal neurodegenerative disease in sheep and goats that occurs naturally and belongs to the group of disorders known as tranmissible spongiform encephalopathies (TSE) or Prion diseases. Prion diseases manifest as infectious, sporadic and/or inherited disorders. They are characterised by the accumulation of protease-resistant isoform of the host encoded prion protein (P...

Prion protein (PrP), which is involved in the pathogenesis of scrapie, occurs in 2 forms. The form extracted from scrapie brain is protease resistant (PrP-res), whereas PrP from normal brain is protease sensitive (PrP-sen). This study examined whether PrP-res could be detected in brains of sheep with scrapie by immunohistochemistry (IHC). A suitable IHC procedure was developed using brain tissu...

The 263K strain of scrapie in hamsters (1,2) has become the most important system for experimental research aimed at discovering the slow unconventional agent which natural ly causes the chronic subacute spongiform encephalopathy 'scrapie' in sheep (3). Other unidenti f ied but similar agents cause similar diseases in man (#), mink (3), and captive mule deer (5). In 1981 P. Merz et al. for the ...

Despite intensive studies on sheep scrapie, a number of questions remain unanswered, such as the natural mode of transmission and the amount of infectivity which accumulates in edible tissues at different stages of scrapie infection. Studies using the mouse model proved to be useful for recognizing scrapie strain diversity, but the low sensitivity of mice to some natural scrapie isolates hamper...

A growing body of evidence suggests that an altered level or function of the neurotrophic insulin-like growth factor-1 receptor (IGF-1R), which supports neuronal survival, may underlie neurodegeneration. This study has focused on the expression and function of the IGF-1R in scrapie-infected neuroblastoma cell lines. Our results show that scrapie infection induces a 4-fold increase in the level ...

Scrapie is a fatal, neurodegenerative disease of sheep and goats. It is also the earliest known member in the family of diseases classified as transmissible spongiform encephalopathies (TSE) or prion diseases, which includes Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy (BSE), and chronic wasting disease in cervids. The recent revelation of naturally occurring BSE in a g...

Data from 4049 Romanov sheep belonging to a flock affected by natural scrapie were analysed by using survival-analysis techniques. Failure time was defined as the period of time between first exposure to infection and the date that animals left the flock with scrapie signs. Four hundred and forty-seven sheep were identified as 'scrapie animals'. Several models, including level of exposure as a ...