title: Conversion of the BASE prion strain into the BSE strain: the origin of BSE?
abstract: Atypical neuropathological and molecular phenotypes of bovine spongiform encephalopathy (BSE) have recently been identified in different countries. One of these phenotypes, named bovine "amyloidotic" spongiform encephalopathy (BASE), differs from classical BSE for the occurrence of a distinct type of the disease-associated prion protein (PrP), termed PrP(Sc), and the presence of PrP amyloid plaques. Here, we show that the agents responsible for BSE and BASE possess different biological properties upon transmission to transgenic mice expressing bovine PrP and inbred lines of nontransgenic mice. Strikingly, serial passages of the BASE strain to nontransgenic mice induced a neuropathological and molecular disease phenotype indistinguishable from that of BSE-infected mice. The existence of more than one agent associated with prion disease in cattle and the ability of the BASE strain to convert into the BSE strain may have important implications with respect to the origin of BSE and spongiform encephalopathies in other species, including humans.
authors.affiliation: Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta, Milan, Italy.
authors.forename: Raffaella
authors.initials: R
authors.lastname: Capobianco
authors.forename: Cristina
authors.initials: C
authors.lastname: Casalone
authors.forename: Silvia
authors.initials: S
authors.lastname: Suardi
authors.forename: Michela
authors.initials: M
authors.lastname: Mangieri
authors.forename: Claudia
authors.initials: C
authors.lastname: Miccolo
authors.forename: Lucia
authors.initials: L
authors.lastname: Limido
authors.forename: Marcella
authors.initials: M
authors.lastname: Catania
authors.forename: Giacomina
authors.initials: G
authors.lastname: Rossi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Di Fede
authors.forename: Giorgio
authors.initials: G
authors.lastname: Giaccone
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Bruzzone
authors.forename: Ludovico
authors.initials: L
authors.lastname: Minati
authors.forename: Cristiano
authors.initials: C
authors.lastname: Corona
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Acutis
authors.forename: Daniela
authors.initials: D
authors.lastname: Gelmetti
authors.forename: Guerino
authors.initials: G
authors.lastname: Lombardi
authors.forename: Martin H
authors.initials: MH
authors.lastname: Groschup
authors.forename: Anne
authors.initials: A
authors.lastname: Buschmann
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Zanusso
authors.forename: Salvatore
authors.initials: S
authors.lastname: Monaco
authors.forename: Maria
authors.initials: M
authors.lastname: Caramelli
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Tagliavini
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000145:classification / Q000209:etiology* / Q000635:transmission*; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D018621:PrPC Proteins / Q000302:isolation & purification* / Q000378:metabolism; D018620:PrPSc Proteins / Q000302:isolation & purification* / Q000378:metabolism
chemical_list: D018621:PrPC Proteins; D018620:PrPSc Proteins