Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
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DOI：
10.1038/383685a0
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发表时间：
1996-10-24
期刊：
影响因子：
64.8
通讯作者：
Hill, AF
中科院分区：
文献类型：
--
作者：
Collinge, J;Sidle, KCL;Hill, AF
传播性海绵状脑病的菌株通过PrPSc的不同理化性质来区分，PrPSc是朊病毒蛋白的疾病相关同种型，其可以在传播给转基因小鼠时保持。“新变种”克雅氏病（CJD）具有与其他类型的CJD不同的菌株特征，并且与BSE传播给小鼠，家猫和猕猴的菌株特征相似，这与BSE是这种新疾病的来源一致。这里揭示的菌株特征表明朊病毒蛋白本身可能编码疾病表型。
Strains of transmissible spongiform encephalopathies are distinguished by differing physicochemical properties of PrPSc, the disease-related isoform of prion protein, which can be maintained on transmission to transgenic mice. 'New variant' Creutzfeldt-Jakob disease (CJD) has strain characteristics distinct from other types of CJD and which resemble those of BSE transmitted to mice, domestic cat and macaque, consistent with BSE being the source of this new disease. Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype.