Ontology highlight
ABSTRACT:
SUBMITTER: Manson JC
PROVIDER: S-EPMC1171748 | biostudies-other | 1999 Dec
REPOSITORIES: biostudies-other
Manson J C JC Jamieson E E Baybutt H H Tuzi N L NL Barron R R McConnell I I Somerville R R Ironside J J Will R R Sy M S MS Melton D W DW Hope J J Bostock C C
The EMBO journal 19991201 23
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. Inoculation with GSS produced disease in 101LL mice in 288 days. Disease was transmitted fro ...[more]