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