DJ-1 binds to mitochondrial complex I and maintains its activity

DJ-1 binds to mitochondrial complex I and maintains its activity
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DOI:
10.1016/j.bbrc.2009.10.025
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发表时间:
2009-12-18
影响因子:
3.1
通讯作者:
Iguchi-Ariga, Sanae M. M.
Iguchi-Ariga, Sanae M. M.
中科院分区:
生物学4区
文献类型:
--
作者:
Hayashi, Takuya;Ishimori, Chikako;Iguchi-Ariga, Sanae M. M.

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帕金森病(PD)是由神经元细胞死亡引起的,氧化应激和线粒体功能障碍被认为是PD发病的原因。dj - 1是家族性帕金森病PARK7的致病基因产物,在转录调控和抗氧化应激中发挥作用。有人提出了DJ-1可能的线粒体功能。但其确切功能尚不清楚。在本研究中,我们发现DJ-1分别与线粒体复合体1的核和线粒体dna编码亚基NDUFA4和ND1直接结合,并与复合体I共定位,在DJ-1敲低的NIH3T3和HEK293细胞中,复合体I的活性降低。这些发现表明,DJ-1是一个完整的线粒体蛋白,DJ-1在维持线粒体复合体I的活性中起作用。(C) 2009爱思唯尔公司版权所有。
Parkinson's disease (PD) is caused by neuronal cell death, and oxidative stress and mitochondrial dysfunction are thought to be responsible for onset of PD. DJ-I, a causative gene product of a familial form of Parkinson's disease, PARK7, plays roles in transcriptional regulation and anti-oxidative stress. The possible mitochondrial function of DJ-1 has been proposed. but its exact function remains unclear. In this study, we found that DJ-1 directly bound to NDUFA4 and ND1, nuclear and mitochondrial DNA-encoding subunits of mitochondrial complex 1, respectively, and was colocalized with complex I and that complex I activity was reduced in DJ-1-knockdown NIH3T3 and HEK293 cells. These findings suggest that DJ-1 is an integral mitochondrial protein and that DJ-1 plays a role in maintenance of mitochondrial complex I activity. (C) 2009 Elsevier Inc. All rights reserved.