Buneeva O A, Medvedev A E
Institute of Biomedical Chemistry, Russian Academy of Medical Sciences, Moscow, 119121, Russia.
Biochemistry (Mosc). 2006 Aug;71(8):851-60. doi: 10.1134/s0006297906080050.
Parkin is a protein encoded by the corresponding parkin gene. It exhibits ubiquitin-protein ligase activity. In this review, we analyze domain structure, substrate specificity, subcellular localization of parkin, and regulation of its activity. Then we discuss data on the effects of various mutations in the parkin gene on structure and functions of this protein and results obtained with parkin knock-out animals. Better understanding of parkin biochemistry, its compartmentalization, functions, and altered functions would help the development of new approaches for the treatment of both inherited and sporadic cases of Parkinson's disease.
帕金蛋白是由相应的帕金基因编码的一种蛋白质。它具有泛素蛋白连接酶活性。在本综述中,我们分析了帕金蛋白的结构域结构、底物特异性、亚细胞定位及其活性调节。然后我们讨论了帕金基因各种突变对该蛋白结构和功能影响的数据,以及帕金基因敲除动物所获得的结果。更好地理解帕金蛋白的生物化学、其区室化、功能以及功能改变,将有助于开发治疗帕金森病遗传病例和散发病例的新方法。