Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

Lambert, Zoe J.; Greenlee, Justin J.; Cassmann, Eric D.; West Greenlee, M. Heather

doi:10.3390/v13122453

Title: Differential Accumulation of Misfolded Prion Strains in Natural Hosts of Prion Diseases

Journal Article · Tue Dec 07 00:00:00 EST 2021 · Viruses

DOI:https://doi.org/10.3390/v13122453· OSTI ID:1983142

^[1]; Greenlee, Justin J. ^[2];

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^[3]

Iowa State Univ., Ames, IA (United States). College of Veterinary Medicine; US Dept. of Agriculture (USDA), Ames, IA (United States). National Animal Disease Center; Oak Ridge Institute for Science and Education (ORISE), Oak Ridge, TN (United States)
US Dept. of Agriculture (USDA), Ames, IA (United States). National Animal Disease Center
Iowa State Univ., Ames, IA (United States). College of Veterinary Medicine

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of neurodegenerative protein misfolding diseases that invariably cause death. TSEs occur when the endogenous cellular prion protein (PrP^C) misfolds to form the pathological prion protein (PrP^Sc), which templates further conversion of PrP^C to PrP^Sc, accumulates, and initiates a cascade of pathologic processes in cells and tissues. Different strains of prion disease within a species are thought to arise from the differential misfolding of the prion protein and have different clinical phenotypes. Different strains of prion disease may also result in differential accumulation of PrP^Sc in brain regions and tissues of natural hosts. Here, we review differential accumulation that occurs in the retinal ganglion cells, cerebellar cortex and white matter, and plexuses of the enteric nervous system in cattle with bovine spongiform encephalopathy, sheep and goats with scrapie, cervids with chronic wasting disease, and humans with prion diseases. By characterizing TSEs in their natural host, we can better understand the pathogenesis of different prion strains. This information is valuable in the pursuit of evaluating and discovering potential biomarkers and therapeutics for prion diseases.

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Research Organization:: Oak Ridge Institute for Science and Education (ORISE), Oak Ridge, TN (United States)

Sponsoring Organization:: USDOE Office of Science (SC); USDA

Grant/Contract Number:: SC0014664

OSTI ID:: 1983142

Journal Information:: Viruses, Vol. 13, Issue 12; ISSN 1999-4915

Publisher:: MDPICopyright Statement

Country of Publication:: United States

Language:: English

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