Department of Neuroscience, Clinical Neurology, University of Pisa, Pisa, Italy.
Parkinsonism Relat Disord. 2009 Dec;15 Suppl 4:S24-7. doi: 10.1016/S1353-8020(09)70830-2.
In eukaryotic cells intracellular components are mainly degraded by autophagy and the ubiquitin-proteasome system. Autophagy is more flexible compared with the ubiquitin-proteasome system and it is involved in the degradation of long-lived proteins and organelles, such as mitochondria, which cannot be degraded by the ubiquitin-proteasome. Although autophagy is able to compensate for ubiquitin-proteasome dysfunction, the opposite does not occur. Autophagy is frequently involved in neurodegeneration; however, there is no consensus on its role in cell survival, as it can be either neuroprotective or neurotoxic. With respect to dopaminergic neurons, there is evidence that autophagy occurs during damage to substantia nigra neurons such as in Parkinson's disease. Moreover, a variety of inherited forms of Parkinson's disease are characterized by mutated proteins that belong to the autophagy pathway. Inhibition of autophagy precipitates dopaminergic cell death, whereas autophagy activation rescues the death of nigral dopaminergic neurons induced by proteasome inhibitors. Taken together, this evidence suggests that autophagy improves the survival of dopaminergic cells.
在真核细胞中,细胞内成分主要通过自噬和泛素蛋白酶体系统降解。与泛素蛋白酶体系统相比,自噬更加灵活,它参与了长寿命蛋白质和细胞器的降解,如线粒体,这些细胞器不能被泛素蛋白酶体降解。虽然自噬能够补偿泛素蛋白酶体功能障碍,但反之则不行。自噬经常与神经退行性变有关;然而,它在细胞存活中的作用尚无定论,因为它既可以是神经保护的,也可以是神经毒性的。就多巴胺能神经元而言,有证据表明,自噬发生在黑质神经元损伤中,如帕金森病。此外,多种遗传性帕金森病的特征是属于自噬途径的突变蛋白。自噬的抑制会促使多巴胺能神经元死亡,而自噬的激活则可以挽救蛋白酶体抑制剂诱导的黑质多巴胺能神经元的死亡。综上所述,这些证据表明自噬可以提高多巴胺能细胞的存活率。