Conversion of the BASE prion strain into the BSE strain: the origin of BSE?
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.
Capobianco
Raffaella
R
Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta, Milan, Italy.
Casalone
Cristina
C
Suardi
Silvia
S
Mangieri
Michela
M
Miccolo
Claudia
C
Limido
Lucia
L
Catania
Marcella
M
Rossi
Giacomina
G
Di Fede
Giuseppe
G
Giaccone
Giorgio
G
Bruzzone
Maria Grazia
MG
Minati
Ludovico
L
Corona
Cristiano
C
Acutis
Pierluigi
P
Gelmetti
Daniela
D
Lombardi
Guerino
G
Groschup
Martin H
MH
Buschmann
Anne
A
Zanusso
Gianluigi
G
Monaco
Salvatore
S
Caramelli
Maria
M
Tagliavini
Fabrizio
F
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
D018621:PrPC Proteins; D018620:PrPSc Proteins