title: [Prions, epidemic of Creutzfeldt-Jakob variant disease and global emergency].
abstract: We present here the current understanding of "prion" theory and global risk for epidemics of variant Creutzfeldt-Jakob disease (vCJD). Prion is the infectious agent of all transmissible spongiform encephalopaties (TSEs). It is regarded as an aggregate of a pathological conformer (PrPSc) of a normal cellular glycoprotein (PrPC) encoded by a gene, in humans on chromosome 20. The differences between PrPSc and PrPC are largely if not exclusively conformational; PrPC is mostly alpha-helical while PrPSc, beta-pleeted. Furthermore, mutations within the coding sequence (open reading frame) of PrP gene are linked with phenotypic expression of CJD, Gerstmann-Straussler-Scheinker disease (GSS) and fatal familial insomnia (FFI). VCJD is caused by transmission from cattle infected with bovine spongiform encephalopathy (BSE). PrP purified from vCJD-affected brains is characterised by the so called fourth type of glycosylation pattern. The neuropathological hallmark of vCJD is the florid plaque, an amyloid plaque surrounded by a corona of spongiform change. VCJD affects mostly young people (range: 13-74 years) and it is clinically distinguishable from sporadic CJD. The extent of epidemics is currently unknown; but its dynamic (102 cases until July 2001) may suggest "the worst" scenario.
authors.affiliation: Zakład Biologii Molekularnej Katedry Biologii Molekularnej, Biochemii i Biofarmacji Slaskiej Akademii Medycznej w Katowicach.
authors.forename: J
authors.initials: J
authors.lastname: Bratosiewicz
authors.forename: P P
authors.initials: PP
authors.lastname: Liberski
mesh_terms: D000682:Amyloid / Q000235:genetics; D003062:Codon; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000453:epidemiology* / Q000209:etiology*; D004252:DNA Mutational Analysis; D019353:Endemic Diseases; D015870:Gene Expression / Q000235:genetics; D006801:Humans; D015994:Incidence; D008967:Molecular Biology / Q000379:methods; D009474:Neurons / Q000473:pathology; D017354:Point Mutation / Q000235:genetics; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics / Q000472:pathogenicity*; D011498:Protein Precursors / Q000235:genetics
chemical_list: D000682:Amyloid; D003062:Codon; C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions; D011498:Protein Precursors