Studies were conducted to determine whether accumulation of the scrapie agent protein Sp33-37 in brain correlated with the appearance of the scrapie agent or with pathology. The concentrations of the scrapie agent and Sp33-37 were measured in purified fraction P5 isolated from hamster brains at weekly intervals after inoculation. The scrapie agent concentration in fraction P5 was approximately ...

BACKGROUND A clear association of amino acid variation in the prion protein gene (PRNP) with susceptibility and resistance to classical scrapie exists in sheep, but not in goats. In this study we examined DNA sequence variation in the PRNP of 149 animals from two scrapie-infected herds of Saanen dairy goats, and identified 6 non-synonymous variants in the coding region. RESULTS In the larger ...

In scrapie-affected sheep flocks, host PrP genotype plays a vital role in determining which sheep will succumb to scrapie and the incubation period. Consequently, within-flock scrapie dynamics is best understood within the context of the genotype profile of the flock. Here we describe a 17 month epidemic of scrapie in a commercially farmed flock of 230 genotyped Texel sheep. At the start of the...

Determinations of scrapie activity in subcellular fractions from infected hamster brains through the asymptomatic and symptomatic course of infection revealed the presence of substantial amounts of scrapie infectivity in the 100,000 X g supernatant fractions, indicating that association with physically discernible membrane structures is not necessary for the transmission of the scrapie agent. A...

This report describes the genetics of the prion protein gene (PRNP) at codons 136, 154, and 171 for sheep diagnosed with naturally acquired classical scrapie in Canada between 1998 and 2008. Genotyping analysis was performed on 249 sheep with confirmed classical scrapie infection representing 98 flocks from 6 provinces. A further case-control analysis of 3 of these flocks compared the genotypes...

Transmissible mink encephalopathy (TME) is a rare disease of ranch-raised mink caused by exposure to an as yet unidentified contaminated food ingredient in the ration. The clinical and pathological similarities between TME and scrapie, together with the indistinguishable physicochemical characteristics of their transmissible agents, suggest that sheep may be the source of infection. However, ex...

In sheep, scrapie susceptibility is so strongly associated with single nucleotide polymorphisms (SNPs) in the gene encoding the prion protein (PrP) that this linkage constitutes the basis for selective breeding strategies directed toward controlling the disease. For goats, in contrast, the association between scrapie susceptibility/resistance and variations in the PrP gene is far weaker, with o...

The neuronal cell damage caused by inducible nitric oxide synthase (iNOS) in brain has been reported to be associated, at least in part, with many neurodegenerative diseases including Alzheimer's disease. We recently observed vacuolation and astrocytosis in the brains of ME7 scrapie strain-infected C57BL mice. To investigate if these phenomena might have a relationship to iNOS, the level of iNO...

BACKGROUND Domestic goats (Capra hircus) are a natural and experimental host of scrapie and bovine spongiform encephalopathy, the transmissible spongiform encephalopathies (TSE) of sheep and cattle. Goats are also susceptible to experimental infection with the agents of TSEs of deer and elk (chronic wasting disease) and humans (Creutzfeldt Jakob disease). Distribution of PrPSc, the abnormal pri...

The amount of incorporation of [5-3H]uridine into polyadenylated RNA was also investigated, 18h after intraperitoneal injection of precursor, in liver and spleen of mice during the late clinical stage of scrapie. Under these conditions the amount of incorporation into polyadenylated RNA in scrapie-infected mice was about 30 % of the normal value in both liver and spleen. In contrast with the re...