NR AJTL
AU Puoti,G.; Giaccone,G.; Rossi,G.; Canciani,B.; Bugiani,O.; Tagliavini,F.
TI Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrPsc in the same brain.
QU Neurology 1999 Dec 10; 53(9): 2173-6
KI Neurology. 1999 Dec 10;53(9):1903-4. PMID: 10599755
PT journal article
AB Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the protease-resistant form of the prion protein (type 1 and type 2 PrPsc). We investigated 14 cases of sporadic CJD and found that both type 1 and type 2 PrPsc coexisted in 5 subjects. The distinct PrPsc isoforms were associated with different patterns of PrP deposition and severity of spongiform changes, suggesting that the PrPsc type plays a central role in determining the neuropathologic profile of CJD.
MH Aged; Blotting, Western; Brain/*pathology/virology; Codon/genetics; Creutzfeldt-Jakob Syndrome/*genetics/pathology/virology; Female; Gene Expression Regulation, Viral/physiology; Human; Male; Middle Age; Phenotype; PrPsc Proteins/*genetics; Prions/*genetics; Support, Non-U.S. Gov't
AD Istituto Nazionale Neurologico Carlo Besta, Milan, Italy.
SP englisch
PO USA