title: Broadening spectrum of bovine spongiform encephalopathies.
abstract: Until recently the etiology of bovine spongiform encephalopathy (BSE) was considered uniform. The infectious agent was thought to be a single strain of prion (posttranslationally altered form of normal prion protein: PrPSc) retaining its biochemical and biological characteristics during interspecies transmission. However, alternate PrPSc signatures through large-scale screening have recently been detected. In addition, genetic alterations governing susceptibility to prion infection and a mutation (E211K) capable of eliciting spontaneous BSE have been demonstrated. Thus, the spectrum of BSEs have broadened and three PrPSc variants (BSE-C, BSE-H and BSE-L) are now defined. Moreover, a new condition resembling BSE, idiopathic brainstem neuronal chromatolysis (IBNC), has been described that may also turn out to be a prion disease. Since one of the new BSE variants, L-type BSE, proved highly pathogenic detection and further characterization of the new conditions are essential.
authors.affiliation: Institute for Medical Microbiology, Semmelweis University, Nagyvárad tér 4, H-1089 Budapest, Hungary. fuzmik@net.sote.hu
authors.forename: M
authors.initials: M
authors.lastname: Füzi
authors.forename: P
authors.initials: P
authors.lastname: Horváth
mesh_terms: D000818:Animals; D001933:Brain Stem / Q000473:pathology; D002417:Cattle; D016643:Encephalopathy, Bovine Spongiform / Q000235:genetics* / Q000473:pathology / Q000635:transmission*; D020022:Genetic Predisposition to Disease; D014644:Genetic Variation; D006801:Humans; D018620:PrPSc Proteins / Q000235:genetics*
chemical_list: D018620:PrPSc Proteins