Mitochondrial complex I and IV activities in leukocytes from patients with parkin mutations
Mitochondrial complex I and IV activities in leukocytes from patients with parkin mutations
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DOI：
10.1002/mds.10695
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发表时间：
2004-05-01
影响因子：
8.6
通讯作者：
Özer, N
中科院分区：
文献类型：
--
作者：
Müftüoglu, M;Elibol, B;Özer, N
parkin蛋白是一种环型泛素蛋白连接酶。考虑到泛素-蛋白体系统活性受损可能会损害抗氧化防御并增强氧化应激，我们研究了帕金基因突变患者线粒体呼吸酶的活性。与年龄匹配的对照组相比，帕金突变患者（62.5%）和特发性PD患者（64.5%）的白细胞复合体I活性均显著降低（P < 0.001）。在特发性PD患者中复合物IV活性也显著降低（60%），但在对照组和帕金突变患者之间没有发现差异。(C) 2003运动障碍学会。
The parkin protein functions as a RING-type ubiquitin protein ligase. Considering the possibility that impaired ubiquitin-proteosomal system activity may impair antioxidant defenses and enhance oxidative stress, we have investigated the activity of mitochondrial respiratory enzymes in patients with parkin gene mutations. A significant decrease in the leukocyte complex I activity was found both in patients with parkin mutations (62.5%) and idiopathic PD (64.5%) compared with age-matched controls (P < 0.001). Complex IV activity was also decreased significantly in idiopathic PD patients (60%), but no difference was detected between controls and patients with parkin mutations. (C) 2003 Movement Disorder Society.