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Article

Effect of the E200K mutation on prion protein metabolism. Comparative study of a cell model and human brain.

The American journal of pathology - 1 Aug 2000

Capellari S, Parchi P, Russo C M, Sanford J, Sy M S, Gambetti P, Petersen R B

Abstract excerpt

The hallmark of prion diseases is the cerebral accumulation of a conformationally altered isoform (PrP(Sc)) of a normal cellular protein, the prion protein (PrP(C)). In the inherited form, mutations in the prion protein gene are thought to cause the disease by altering the metabolism of the mutant PrP (PrP(M)) engendering its conversion into PrP(Sc). We used a cell model to study biosynthesis and processing of...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 10934164
DOI: 10.1016/S0002-9440(10)64572-5
PMCID: PMC1850141

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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