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J Biol Chem. 2014 Jul 18;289(29):19841-9. doi: 10.1074/jbc.R114.550707. Epub 2014 May 23.

Mouse models for studying the formation and propagation of prions.

Author information

1
From the Institute for Neurodegenerative Diseases and the Department of Neurology, University of California, San Francisco, California 94143.
2
From the Institute for Neurodegenerative Diseases and the Department of Neurology, University of California, San Francisco, California 94143 stanley@ind.ucsf.edu.

Abstract

Prions are self-propagating protein conformers that cause a variety of neurodegenerative disorders in humans and animals. Mouse models have played key roles in deciphering the biology of prions and in assessing candidate therapeutics. The development of transgenic mice that form prions spontaneously in the brain has advanced our understanding of sporadic and genetic prion diseases. Furthermore, the realization that many proteins can become prions has necessitated the development of mouse models for assessing the potential transmissibility of common neurodegenerative diseases. As the universe of prion diseases continues to expand, mouse models will remain crucial for interrogating these devastating illnesses.

KEYWORDS:

Animal Model; Neurodegenerative Disease; Pathology; Prion; Protein Aggregation

PMID:
24860095
PMCID:
PMC4106304
DOI:
10.1074/jbc.R114.550707
[Indexed for MEDLINE]
Free PMC Article

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