From genetics to pathology:: tau and α-synuclein assemblies in neurodegenerative diseases
From genetics to pathology:: tau and α-synuclein assemblies in neurodegenerative diseases
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DOI：
10.1098/rstb.2000.0767
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发表时间：
2001-02-28
期刊：
影响因子：
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通讯作者：
Crowther, RA
中科院分区：
文献类型：
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作者：
Goedert, M;Spillantini, MG;Crowther, RA
人脑最常见的退行性疾病的特征在于在受影响的神经细胞和神经胶质细胞中存在异常丝状包涵体。这些疾病可以分为两类，基于丝状组件的主要蛋白质组分的身份。这些细丝由微管相关蛋白tau或α-突触核蛋白组成。重要的是，在家族性额颞叶痴呆中tau基因突变和在家族性帕金森病中α-突触核蛋白基因突变的发现已经证实tau蛋白和α-突触核蛋白的功能障碍可以引起神经变性.
The most common degenerative diseases of the human brain are characterized by the presence of abnormal filamentous inclusions in affected nerve cells and glial cells. These diseases can be grouped into two classes, based on the identity of the major proteinaceous components of the filamentous assemblies. The filaments are made of either the microtubule-associated protein tau or the protein alpha -synuclein. Importantly, the discovery of mutations in the tau gene in familial forms of frontotemporal dementia and of mutation in the alpha -synuclein gene in familial forms of Parkinson's disease has established that dysfunction of tau protein and alpha -synuclein can cause neurodegeneration.