Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain.
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 PrP(Sc)). We investigated 14 cases of sporadic CJD and found that both type 1 and type 2 PrP(Sc) coexisted in 5 subjects. The distinct PrP(Sc) isoforms were associated with different patterns of PrP deposition and severity of spongiform changes, suggesting that the PrP(Sc) type plays a central role in determining the neuropathologic profile of CJD.
Puoti
G
G
Istituto Nazionale Neurologico Carlo Besta, Milan, Italy.
Giaccone
G
G
Rossi
G
G
Canciani
B
B
Bugiani
O
O
Tagliavini
F
F
D000368:Aged; D015153:Blotting, Western; D001921:Brain / Q000473:pathology* / Q000821:virology; D003062:Codon / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics* / Q000473:pathology / Q000821:virology; D005260:Female; D015967:Gene Expression Regulation, Viral / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D018620:PrPSc Proteins / Q000235:genetics*; D011328:Prions / Q000235:genetics*
D003062:Codon; D018620:PrPSc Proteins; D011328:Prions