Sporadic Creutzfeldt-Jakob disease:: Co-occurrence of different types of PrPSc in the same brain

被引：154

作者：

Puoti, G ^{[1
]}

Giaccone, G ^{[1
]}

Rossi, G ^{[1
]}

Canciani, B ^{[1
]}

Bugiani, O ^{[1
]}

Tagliavini, F ^{[1
]}

机构：

[1] Ist Nazl Neurol Carlo Besta, I-20133 Milan, Italy

来源：

NEUROLOGY | 1999年 / 53卷 / 09期

关键词：

Creutzfeldt-Jakob disease; neuropathology; prion protein; western blot analysis;

D O I：

10.1212/WNL.53.9.2173

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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 I 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.

引用

页码：2173 / 2176

页数：4

共 12 条

[1] DISTINCT PRP PROPERTIES SUGGEST THE MOLECULAR-BASIS OF STRAIN VARIATION IN TRANSMISSIBLE MINK ENCEPHALOPATHY [J].