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Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS

Authors
  • Requena, Jesús R.
Type
Published Article
Journal
PLoS Biology
Publisher
Public Library of Science
Publication Date
Jun 25, 2020
Volume
18
Issue
6
Identifiers
DOI: 10.1371/journal.pbio.3000754
PMID: 32584805
PMCID: PMC7316225
Source
PubMed Central
License
Unknown

Abstract

This Primer explores the implications of a study that describes the first murine model of familial human prion disease, demonstrating the emergence and propagation of two PrP amyloid conformers; of these, one causes neurodegeneration while the other does not. With its many conformers, PrP is a truly protean protein.

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