Lethal recessive myelin toxicity of prion protein lacking its central domain

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Lethal recessive myelin toxicity of prion protein lacking its central domain.

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 ph...

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ژورنال

عنوان ژورنال: The EMBO Journal

سال: 2007

ISSN: 0261-4189,1460-2075

DOI: 10.1038/sj.emboj.7601510