Parkin， Parkin 底物和帕金森病

Parkin 是隐性遗传性少年型帕金森病的致病基因 . 现认为 Parkin 行使泛素蛋白连接酶功能，参与蛋白质的泛素化过程 . 它的功能缺陷致使其底物蛋白质毒性积聚，从而介导多巴胺能神经元选择性死亡 . 越来越多的研究显示 Parkin 还具有神经保护作用，能对抗多种神经毒性刺激，并且可能参与路易体的形成过程，因此认为它在散发性帕金森病的致病过程中也可能起重要作用 .

Parkin, Parkin Substrates and Parkinson's Disease

Parkin is a causative gene of autosomal recessive juvenile parkinsonism. Now it is believed that Parkin functions as an E3 ubiquitin protein ligase which involves in protein's ubiquitination. Parkin defect makes its substrates accumulate, which eventually leads to dopaminergic neuron selective death. More and more evidence shows that Parkin can protect neurons from various neuron toxic stimulations and may participate in the formation of Lewy body, therefore Parkin may be important in the pathogenesis of sporadic Parkinson's disease.