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Article

Variably protease-sensitive prionopathy, a unique prion variant with inefficient transmission properties.

Emerging infectious diseases - 1 Dec 2014

Diack Abigail B, Ritchie Diane L, Peden Alexander H, Brown Deborah, Boyle Aileen, Morabito Laura, Maclennan David, Burgoyne Paul, Jansen Casper, Knight Richard S, Piccardo Pedro, Ironside James W, Manson Jean C

Abstract excerpt

Variably protease-sensitive prionopathy (VPSPr) can occur in persons of all codon 129 genotypes in the human prion protein gene (PRNP) and is characterized by a unique biochemical profile when compared with other human prion diseases. We investigated transmission properties of VPSPr by inoculating transgenic mice expressing human PRNP with brain tissue from 2 persons with the valine-homozygous (VV) and 1 with the...

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Identifiers

PMID: 25418327
DOI: 10.3201/eid2012.140214
PMCID: PMC4257789

Genes

PRNP

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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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