NR AXZP
AU Zanusso,G.; Lombardi,G.; Casalone,C.; D'Angelo,A.; Gelmetti,D.; Torcoli,G.; Barbieri,I.; Corona,C.; Fasoli,E.; Farinazzo,A.; Fiorini,M.; Gelati,M.; Iulini,B.; Tagliavini,F.; Ferrari,S.; Monaco,S.; Caramelli,M.; Capucci,L.
TI Transmission of Italian BSE and BASE Isolates in Cattle Results into a Typical BSE Phenotype and a Muscle Wasting Disease
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Oral Abstracts FC5.5.2
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Vortrag
AB
The clinical phenotype of bovine spongiform encephalopathy has been extensively reported in early accounts of the disorder. Following the introduction of statutory active surveillance, almost all BSE cases have been diagnosed on a pathological/molecular basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance system has uncovered atypical BSE cases, which are characterized by distinct conformers of the PrPsc, named high-type (BSE-H) and low-type (BSE-L), whose clinicopathological phenotypes remain unknown. We recently reported two Italian atypical cases with a PrPsc type similar to BSE-L, pathologically characterized by PrP amyloid plaques. Experimental transmission to TgBov mice has recently disclosed that BASE is caused by a distinct prion strain which is extremely virulent. A major limitation of transmission studies to mice is the lack of reliable information on clinical phenotype of BASE in its natural host. In the present study, we experimentally infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and BASE isolates by i.c.
route. BASE infected cattle showed survival times significantly shorter than BSE, a finding more readily evident in Fresian/Holstein, and in keeping with previous observations in TgBov mice. Clinically, BSE-infected cattle developed a disease phenotype highly comparable with that described in field BSE cases and in experimentally challenged cattle. On the contrary, BASE-inoculated cattle developed an amyotrophic disorder accompanied by mental dullness.
The molecular and neuropathological profiles, including PrP deposition pattern, closely matched those observed in the original cases. This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.
AD G. Zanusso, E. Fasoli, A. Farinazzo, M. Fiorini, M. Gelati, S. Ferrari, S. Monaco, University of Verona, Neurological and Visual Sciences, Italy; G. Lombardi, D. Gelmetti, G. Torcoli, I. Barbieri, L. Capucci, IZSLER, Italy; C. Casalone, C. Corona, B. Iulini, M. Caramelli, IZSPLVA, Italy; A. D'Angelo, University of Turin, Animal Pathology, Italy; F. Tagliavini, Isituto Carlo Besta, Italy
SP englisch
PO Schottland