Article
A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy.
The EMBO journal - 1 Dec 1999
Manson J C, Jamieson E, Baybutt H, Tuzi N L, Barron R, McConnell I, Somerville R, Ironside J, Will R, Sy M S, Melton D W, Hope J, Bostock C
Abstract excerpt
A mutation equivalent to P102L in the human PrP gene, associated with Gerstmann-Straussler syndrome (GSS), has been introduced into the murine PrP gene by gene targeting. Mice homozygous for this mutation (101LL) showed no spontaneous transmissible spongiform encephalopathy (TSE) disease, but had incubation times dramatically different from wild-type mice following inoculation with different TSE sources....
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