title: Classical bovine spongiform encephalopathy by transmission of H-type prion in homologous prion protein context.
abstract: Bovine spongiform encephalopathy (BSE) and BSE-related disorders have been associated with a single major prion strain. Recently, 2 atypical, presumably sporadic forms of BSE have been associated with 2 distinct prion strains that are characterized mainly by distinct Western blot profiles of abnormal protease-resistant prion protein (PrPres), named high-type (BSE-H) and low-type (BSE-L), that also differed from classical BSE. We characterized 5 atypical BSE-H isolates by analyzing their molecular and neuropathologic properties during transmission in transgenic mice expressing homologous bovine prion protein. Unexpectedly, in several inoculated animals, strain features emerged that were highly similar to those of classical BSE agent. These findings demonstrate the capability of an atypical bovine prion to acquire classical BSE-like properties during propagation in a homologous bovine prion protein context and support the view that the epidemic BSE agent could have originated from such a cattle prion.
authors: Centro de Investigación en Sanidad Animal, Madrid, Spain. jmtorres@inia.es
authors: Juan-María
authors: JM
authors: Torres
authors: Olivier
authors: O
authors: Andréoletti
authors: Caroline
authors: C
authors: Lacroux
authors: Irene
authors: I
authors: Prieto
authors: Patricia
authors: P
authors: Lorenzo
authors: Magdalena
authors: M
authors: Larska
authors: Thierry
authors: T
authors: Baron
authors: Juan-Carlos
authors: JC
authors: Espinosa
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000209:etiology / Q000473:pathology*; D051379:Mice; D008822:Mice, Transgenic; D018621:PrPC Proteins / Q000235:genetics / Q000378:metabolism*; D018620:PrPSc Proteins / Q000302:isolation & purification / Q000472:pathogenicity*; D013154:Spleen / Q000378:metabolism / Q000473:pathology
chemical_list: D018621:PrPC Proteins; D018620:PrPSc Proteins