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首页> 外文期刊>Journal of neurology >Aggregation of alpha-synuclein in the pathogenesis of Parkinson's disease.
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Aggregation of alpha-synuclein in the pathogenesis of Parkinson's disease.

机译：帕金森氏病发病机理中α-突触核蛋白的聚集。

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摘要

Lewy bodies (LBs) are hallmark lesions in the brains of patients with Parkinson's disease (PD) and dementia with Lewy bodies (DLB). By raising a monoclonal antibody LB509 against purified LBs from the brains of patients with DLB that strongly immuola-beled LBs, we found that alpha-synuclein is one of the major components of LBs. Thus, the deposition of alpha-synuclein, an abundant presynaptic brain protein, as fibrillary aggregates in affected neurons or glial cells,was highlighted as a hallmark lesion of a subset of neurodegenerative disorders, including PD, DLB and multiple system atrophy collectively referred to as synucleinopathies. Importantly, the identification of missense mutations in alpha-synuclein gene in some pedigrees of familial PD has strongly implicated alpha-synuclein in the pathogenesis of PD and other synucleinopathies. We then examined the specific post-translational modifications that characterize and underlie the aggregation of alpha-synuclein in synucleinopathy brains by mass spectrometry and using a s pecific antibody,and found that serine 129 of alpha-synuclein deposited in synucleinopathy lesions is selectively and extensively phosphorylated. These findings underscore the importance of phosphorylation of filamentous proteins in the pathogenesis of neurodegenerative disorders.
机译：路易体（LBs）是帕金森氏病（PD）和痴呆伴路易体（DLB）患者大脑中的标志性病变。通过从单克隆抗体LB509中纯化出的LB单克隆抗体LB509产生强烈的免疫印迹，我们发现α-突触核蛋白是LB的主要成分之一。因此，突出显示了α-突触核蛋白（一种丰富的突触前脑蛋白）在受影响的神经元或神经胶质细胞中作为原纤维聚集体的沉积，是神经退行性疾病（包括PD，DLB和多系统萎缩）的子集的标志性病变，统称为突触核病。重要的是，在家族性PD的某些家系中对α-突触核蛋白基因的错义突变的鉴定已将α-突触核蛋白强烈地牵涉到PD和其他突触核蛋白病的发病机理中。然后，我们通过质谱法和使用特异性抗体检查了特定的翻译后修饰，这些修饰后的修饰表征并突显了突触核蛋白脑中α-突触核蛋白的聚集，并发现沉积在突触核蛋白病病变中的α-突触核蛋白的丝氨酸129被选择性地广泛磷酸化。这些发现强调了丝状蛋白磷酸化在神经退行性疾病发病机理中的重要性。

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