NR ABQB
AU Bratosiewicz,J.; Liberski,P.P.
TI [Prions, epidemic of Creutzfeldt-Jakob variant disease and global emergency]
OT Priony, epidemia wariantu choroby Creutzfeldta-Jakoba i zagrozenie globalne
QU Neurologia i Neurochirurgia Polska 2001; 35(4 Suppl): 25-44
PT journal article
AB 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-Sträussler-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.
MH Amyloid/genetics; Codon; Creutzfeldt-Jakob Syndrome/classification/*epidemiology/*etiology; DNA Mutational Analysis; Endemic Diseases; English Abstract; Gene Expression/genetics; Human; Incidence; Molecular Biology/methods; Neurons/pathology; Point Mutation/genetics; Prions/genetics/*pathogenicity; Protein Precursors/genetics
AD Zaklad Biologii Molekularnej Katedry Biologii Molekularnej, Biochemii i Biofarmacji Slaskiej Akademii Medycznej w Katowicach.
SP polnisch
PO Polen