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帕金森病是一种常见的神经系统变性疾病,以黑质多巴胺能神经元进行性缺失及α-synuclein聚集和路易小体(LBs)的形成为主要特点。虽然经历了大量的研究,但PD的病因和发病机制仍不完全清楚。最近,许多证据显示泛素-蛋白酶体系统功能障碍与PD发病机制之间存在关系。泛素-蛋白酶体系统可清除体内突变、损伤和异常折叠的蛋白质,起到调控蛋白质,维持细胞内环境稳定的作用,一旦这种功能受抑,平衡被打破,就会引起这些异常蛋白质在细胞内聚集、沉积、LBs形成,最终导致多巴胺能神经元变性坏死。本文就泛素-蛋白酶体系统与帕金森病发病机制的关系做一综述。
Parkinson’s disease is a common degenerative disease of the nervous system characterized by the progressive loss of substantia nigra dopaminergic neurons and the accumulation of α-synuclein and the formation of Lewy bodies (LBs). Although undergoing a large number of studies, but the etiology and pathogenesis of PD is still not fully understood. Recently, there is much evidence that there is a relationship between the dysfunction of the ubiquitin-proteasome system and the pathogenesis of PD. Ubiquitin - proteasome system can clear the body of mutations, damage and abnormal folding of the protein, play a regulatory role in the protein to maintain the stability of the role of intracellular environment, once this function is suppressed, the balance is broken, it will cause these abnormal proteins in the cells Accumulation, deposition, LBs formation, eventually leading to degeneration and necrosis of dopaminergic neurons. This article reviews the relationship between the ubiquitin-proteasome system and the pathogenesis of Parkinson’s disease.