title: Molecular barriers to zoonotic transmission of prions.
abstract: The risks posed to human health by individual animal prion diseases cannot be determined a priori and are difficult to address empirically. The fundamental event in prion disease pathogenesis is thought to be the seeded conversion of normal prion protein to its pathologic isoform. We used a rapid molecular conversion assay (protein misfolding cyclic amplification) to test whether brain homogenates from specimens of classical bovine spongiform encephalopathy (BSE), atypical BSE (H-type BSE and L-type BSE), classical scrapie, atypical scrapie, and chronic wasting disease can convert normal human prion protein to the abnormal disease-associated form. None of the tested prion isolates from diseased animals were as efficient as classical BSE in converting human prion protein. However, in the case of chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
authors.forename: Marcelo A
authors.initials: MA
authors.lastname: Barria
authors.forename: Aru
authors.initials: A
authors.lastname: Balachandran
authors.forename: Masanori
authors.initials: M
authors.lastname: Morita
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto
authors.forename: Rona
authors.initials: R
authors.lastname: Barron
authors.forename: Jean
authors.initials: J
authors.lastname: Manson
authors.forename: Richard
authors.initials: R
authors.lastname: Knight
authors.forename: James W
authors.initials: JW
authors.lastname: Ironside
authors.forename: Mark W
authors.initials: MW
authors.lastname: Head
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D004198:Disease Susceptibility; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D017096:Prion Diseases / Q000235:genetics / Q000635:transmission*; D011328:Prions / Q000235:genetics / Q000378:metabolism*; D012756:Sheep; D015047:Zoonoses / Q000235:genetics / Q000635:transmission*
chemical_list: D011328:Prions
keywords: BSE; CJD; Creutzfeldt-Jakob disease; bovine spongiform encephalopathy; cell-free system; chronic wasting disease; in vitro assay; prion disease; prions; protein misfolding diseases; scrapie; zoonoses