MITOCHONDRIAL RESPIRATORY-CHAIN ACTIVITY IN SKELETAL-MUSCLE FROM PATIENTS WITH PARKINSONS-DISEASE
MITOCHONDRIAL RESPIRATORY-CHAIN ACTIVITY IN SKELETAL-MUSCLE FROM PATIENTS WITH PARKINSONS-DISEASE
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DOI：
10.1212/wnl.43.11.2258
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发表时间：
1993-11-01
期刊：
影响因子：
9.9
通讯作者：
URBANOMARQUEZ, A
中科院分区：
文献类型：
--
作者：
CARDELLACH, F;MARTI, MJ;URBANOMARQUEZ, A
在特发性帕金森病（PD）患者的肌肉和其他组织中，线粒体电子传递链活性存在不同的异常情况。我们研究了8名西班牙帕金森病患者，以评估该国患者肌肉线粒体中电子传递链的功能活性。我们发现患者的复合物I活性（nmol·min⁻¹·mg⁻¹）（245.8 ± 42.8）低于对照组（331.6 ± 60.1）（p = 0.004），患者的复合物IV活性（46.1 ± 9）低于对照组（144.1 ± 42.3）（p = 0.00001）。两名患者的复合物V活性也有所降低，所有患者的复合物II和III活性均正常。尽管这些结果强烈表明帕金森病中线粒体DNA发生了改变，但不同组织中各种电子传递链缺陷似乎是非特异性的。
Different abnormalities in mitochondrial electron transport chain activity have been demonstrated in muscle and other tissues of patients with idiopathic Parkinson's disease (PD). We studied eight Spanish patients with PD to evaluate the functional activity of the electron transport chain in muscle mitochondria from patients of this country. We found lower complex I activity (nmol.min-1.mg-1) in patients (245.8 +/- 42.8) than in controls (331.6 +/- 60.1) (p = 0.004) and lower complex IV activity in patients (46.1 +/- 9) than in controls (144.1 +/- 42.3) (p = 0.00001). Complex V activity was also decreased in two patients and complex II and III activities were normal in all of them. Although these results strongly suggest an alteration in mitochondrial DNA in PD, the various electron transport chain defects in different tissues seem to be nonspecific.