NR AWUC
AU Capobianco,R.; Casalone,C.; Suardi,S.; Mangieri,M.; Miccolo,C.; Limido,L.; Catania,M.; Rossi,G.; Di Fede,G.; Giaccone,G.; Bruzzone,M.G.; Minati,L.; Corona,C.; Acutis,P.L.; Gelmetti,D.; Lombardi,G.; Groschup,M.H.; Buschmann,A.; Zanusso,G.; Monaco,S.; Caramelli,M.; Tagliavini,F.
TI Conversion of the BASE prion strain into the BSE strain: the origin of BSE?
QU PLoS Pathogens 2007 Mar 9; 3(3): e31
IA http://pathogens.plosjournals.org/perlserv/?request=get-document&doi=10.1371%2Fjournal.ppat.0030031
PT journal article; research support, non-u.s. gov't
AB 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 PrPsc, 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.
MH Animals; Brain/metabolism; Cattle; Encephalopathy, Bovine Spongiform/classification/*etiology/*transmission; Mice; Mice, Inbred C57BL; Mice, Transgenic; PrPc Proteins/*isolation & purification/metabolism; PrPsc Proteins/*isolation & purification/metabolism
AD Raffaella Capobianco, Silvia Suardi, Michela Mangieri, Claudia Miccolo, Lucia Limido, Marcella Catania, Giacomina Rossi, Giuseppe Di Fede, Giorgio Giaccone, Maria Grazia Bruzzone, Ludovico Minati, Fabrizio Tagliavini, Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta, Milan, Italy; Cristina Casalone, Cristiano Corona, Pierluigi Acutis, Maria Caramelli, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Torino, Italy; Daniela Gelmetti, Guerino Lombardi, Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Brescia, Italy; Martin H. Groschup, Anne Buschmann, Friedrich-Loeffler-Institut, Institute for Novel and Emerging Infectious Diseases, Greifswald, Insel Riems, Germany; Gianluigi Zanusso, Salvatore Monaco, Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G. B. Rossi, Verona, Italy
SP englisch
PO USA