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α-突触核蛋白(AS)是Lewy体的重要组成成分。AS基因定位于第4号染色体,其突变型与常染色体显性遗传性帕金森病(PD)的发病密切相关。在PD中,AS出现了折叠错误和排列混乱。AS的聚集能力,特别是在氧化应激状态下,被认为是其病理机制的核心,AS的异常聚集和降解障碍导致蛋白酶的抑制和多巴胺能神经元的死亡。因此,AS致病形式对认识PD的发生有重要意义。
Alpha-synuclein (AS) is an important component of Lewy bodies. The AS gene is located on chromosome 4, and its mutation is closely related to the onset of autosomal dominant Parkinson’s disease (PD). In the PD, the AS appeared to be folded and misconfigured. The ability of AS to aggregate, especially in oxidative stress, is thought to be central to its pathological mechanisms, and aberrant aggregation and degradation of AS lead to protease inhibition and dopaminergic neuron death. Therefore, the pathogenic form of AS is of great importance to recognize the occurrence of PD.