NR AWOO
AU Tagliavini,F.; Capobianco,R.; Casalone,C.; Suardi,S.; Mangieri,M.; Miccolo,C.; Limido,L.; Catania,M.; Rossi,G.; Giaccone,G.; Corona,C.; Gelmetti,D.; Lombardi,G.; Groschup,M.H.; Buschmann,A.; Zanusso,G.; Monaco,S.; Caramelli,M.
TI Biological properties of the prion strain linked to the amyloidotic form of bovine spongiform encephalopathy (BASE)
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Oral sessions ORAL-11
PT Konferenz-Vortrag
AB In the past few years, two atypical forms of bovine spongiform encephalopathy (BSE) have been recognized in different European countries, Japan and USA through active surveillance systems. One of these phenotypes has been identified in Italy and is distinguishable from classical BSE for remarkable differences in pattern of deposition and brain regional distribution of PrPsc, with presence of PrP-immunoreactive amyloid plaques and severe involvement of the olfactory system with relative sparing of the brainstem. The molecular signature of this "amyloidotic" form of bovine spongiform encephalopathy, named BASE, is a PrPsc type having a protease-resistant core of lower molecular mass than BSE-PrPsc with predominance of the monoglycosylated species. We carried out strain typing studies using transgenic mice expressing bovine PrP (Tg Bov mice) and inbred lines of nontransgenic mice, including SJL, C57Bl/6, RIII and VM mice. Both BSE and BASE transmitted readily to Tg Bov mice, and produced different clinical, neuropathological and molecular disease phenotypes indicating the propagation of two distinct prion strains. Conversely, all inbred mouse lines showed a substantial barrier to primary transmission of BASE. Unexpectedly, second-passage transmission of the BASE strain to non-transgenic 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.
AD F. Tagliavini, R. Capobianco, S.Suardi, M. Mangieri, C. Miccolo, L. Limido, M. Catania, G. Rossi, G. Giaccone: Istituto Nazionale Neurologico Carlo Besta, Milan, Italy; C. Casalone, C. Corona, M. Caramelli: Istituto Zooprofilattico Sperimentale del Piemonte, Liguria i Valle dAosta, Torino, Italy; D. Gelmetti, G. Lombardi: Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Brescia, Italy; M.H. Groschup, A. Buschmann: Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany; G. Zanusso, S. Monaco: Department of Neurological and Visual Science, University of Verona, Italy
SP englisch
PO Italien