Ontology highlight
ABSTRACT:
SUBMITTER: Baumann F
PROVIDER: S-EPMC1783444 | biostudies-literature | 2007 Jan
REPOSITORIES: biostudies-literature
Baumann Frank F Tolnay Markus M Brabeck Christine C Pahnke Jens J Kloz Ulrich U Niemann Hartmut H HH Heikenwalder Mathias M Rülicke Thomas T Bürkle Alexander A Aguzzi Adriano A
The EMBO journal 20070101 2
PrP(C)-deficient mice expressing prion protein variants with large amino-proximal deletions (termed PrP(DeltaF)) suffer from neurodegeneration, which is rescued by full-length PrP(C). We now report that expression of PrP(DeltaCD), a PrP variant lacking 40 central residues (94-134), induces a rapidly progressive, lethal phenotype with extensive central and peripheral myelin degeneration. This phenotype was rescued dose-dependently by coexpression of full-length PrP(C) or PrP(C) lacking all octare ...[more]