NR AERU
AU Goldmann,W.; Hunter,N.; Smith,G.; Foster,J.; Hope,J.
TI PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie.
QU Journal of General Virology 1994 May; 75(5): 989-95
PT journal article
AB Man and sheep are the two species in which spongiform encephalopathies occur naturally, and in which there are recognized genetic components that predispose an individual person or sheep to clinical disease. In both species mutations/polymorphisms in the PrP gene have been linked to the incidence of natural disease, but only in sheep is it possible to investigate by deliberate exposure to infection whether these polymorphisms are directly correlated with survival time. Cheviot sheep of different PrP genotypes were challenged with one of two isolates of scrapie or an isolate of bovine spongiform encephalopathy and the survival time and incidence of disease were monitored. Genotype analysis showed that dimorphisms in codons 136 and 171 of the ovine PrP gene correlated with control of disease incidence and modulation of incubation time. Crucially, the functional effects of these domains of PrP were shown to alternate depending on the isolate of infecting agent.
IN Genotypanalysen haben gezeigt, dass Dimorphismen in den Codons 136 und 171 Prionproteingens im Schaf mit der Kontrolle des Krankheitsausbruches und der Modulation der Inkubationszeit korreliert. Dabei hängt die Wirkung der verschiedenen Prionproteinallele von dem jeweiligen infektiösen Agens ab.
MH Alleles; Animal; Base Sequence; Cattle; Codon; Comparative Study; Encephalopathy, Bovine Spongiform; Genotype; Molecular Sequence Data; PrPsc Proteins; Prion Diseases/*etiology; Prions/administration & dosage/*genetics; Scrapie/*etiology; Sheep/*genetics; Structure-Activity Relationship; Support, Non-U.S. Gov't; Survival Analysis; Variation (Genetics)
AD Institute for Animal Health, AFRC and MRC Neuropathogenesis Unit, Edinburgh, U.K.
SP englisch
PO England
OR Prion-Krankheiten G