title: Limited amplification of chronic wasting disease prions in the peripheral tissues of intracerebrally inoculated cattle.
abstract: Chronic wasting disease (CWD) is a fatal neurodegenerative disease, classified as a prion disease or transmissible spongiform encephalopathy (TSE) similar to bovine spongiform encephalopathy (BSE). Cervids affected by CWD accumulate an abnormal protease-resistant prion protein throughout the central nervous system (CNS), as well as in both lymphatic and excretory tissues - an aspect of prion disease pathogenesis not observed in cattle with BSE. Using seeded amplification through real-time quaking-induced conversion, we investigated whether the bovine host or prion agent was responsible for this aspect of TSE pathogenesis. We blindly examined numerous central and peripheral tissues from cattle inoculated with CWD for prion seeding activity. Seeded amplification was readily detected in the CNS, though rarely observed in peripheral tissues, with a limited distribution similar to that of BSE prions in cattle. This seems to indicate that prion peripheralization in cattle is a host-driven characteristic of TSE infection.
authors.affiliation: Department of Basic Sciences, Midwestern University, Glendale, AZ, USA.|Department of Diagnostic Medicine and Pathobiology, College of Veterinary Medicine, Kansas State University, Manhattan, KS, USA.
authors.forename: Nicholas J
authors.initials: NJ
authors.lastname: Haley
authors.affiliation: Department of Diagnostic Medicine and Pathobiology, College of Veterinary Medicine, Kansas State University, Manhattan, KS, USA.
authors.forename: Christopher
authors.initials: C
authors.lastname: Siepker
authors.affiliation: Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, Ames, Iowa, USA.
authors.forename: Justin J
authors.initials: JJ
authors.lastname: Greenlee
authors.affiliation: Department of Diagnostic Medicine and Pathobiology, College of Veterinary Medicine, Kansas State University, Manhattan, KS, USA.
authors.forename: Jürgen A
authors.initials: JA
authors.lastname: Richt
mesh_terms: D000818:Animals; D002417:Cattle / Q000378:metabolism; D002490:Central Nervous System / Q000473:pathology*; D003670:Deer / Q000378:metabolism*; D016643:Encephalopathy, Bovine Spongiform / Q000473:pathology; D000072002:Prion Proteins / Q000378:metabolism*; D011328:Prions / Q000378:metabolism*; D017510:Protein Folding; D034081:Wasting Disease, Chronic / Q000473:pathology* / Q000635:transmission*
chemical_list: D000072002:Prion Proteins; D011328:Prions