Sporadic Creutzfeldt-Jakob disease:: Co-occurrence of different types of PrPSc in the same brain
Sporadic Creutzfeldt-Jakob disease:: Co-occurrence of different types of PrPSc in the same brain
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DOI：
10.1212/wnl.53.9.2173
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发表时间：
1999-12-10
期刊：
影响因子：
9.9
通讯作者：
Tagliavini, F
中科院分区：
文献类型：
--
作者：
Puoti, G;Giaccone, G;Tagliavini, F
散发性克雅氏病 (CJD) 的表型异质性与生化上不同类型的蛋白酶抗性朊病毒蛋白类型（1 型和 2 型 PrPSc）有关。我们调查了 14 例散发性克雅氏病，发现 5 名受试者同时存在 I 型和 2 型 PrPSc。不同的 PrPSc 亚型与不同的 PrP 沉积模式和海绵状变化的严重程度相关，表明 PrPSc 类型在确定克雅氏病的神经病理学特征中起着核心作用。
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.