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. 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

Free PMC article

Mouse Models for Studying the Formation and Propagation of Prions

Joel C Watts et al. J Biol Chem. .
Free PMC article


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.


Neuropathological features of prion disease in mice. Brains from clinically ill wild-type mice that were intracerebrally inoculated with the RML strain of mouse-passaged scrapie prions (left column) or spontaneously ill Tg mice expressing the Ile-109 polymorphic variant of bank vole PrP (right column) exhibit all of the neuropathological hallmarks of prion disease. These characteristics include spongiform degeneration (black arrows), as revealed by H&E staining; deposition of aggregated and misfolded prion protein (red arrows), as revealed by immunolabeling for PrPSc; and astrocytic gliosis, as revealed by immunostaining for GFAP.

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