Benecke R, Strümper P, Weiss H
Neurologische Klinik, Heinrich-Heine-Universität, Düsseldorf, Germany.
Brain. 1993 Dec;116 ( Pt 6):1451-63. doi: 10.1093/brain/116.6.1451.
Using a technique which requires only 100 ml blood we investigated the electron transfer complexes (ETC) I, III and IV in platelet mitochondria of 44 control subjects, 27 patients with idiopathic Parkinson's disease and eight patients with Parkinson-plus syndromes due to multiple system atrophy. In both control subjects and patients, ETC measurements were repeated at intervals of several months. The activities varied considerably among normal subjects, but intra-individual variation of ETC activities were low at repetitive measurements. In normal subjects there was no correlation between enzyme activities and age or training state. There was no difference in enzyme activities between smokers and non-smokers in the control group. Complex I activity was lower in Parkinson's disease patients than in controls (14 versus 29 nmol/min/mg platelet protein; P < 0.001). Furthermore, the group difference in complex IV activity also reached statistical significance (83 versus 58 nmol/min/mg platelet protein; P < 0.001). Additionally, in some Parkinson's disease patients, activities of complex III were low and lay outside the control range, but the group difference did not reach significance. There was no correlation between complex I activity and disease duration or severity as well as the daily L-dopa dose in Parkinson's disease patients. Repeated measurements in five Parkinson's disease patients in the earliest stages of their illness demonstrated that the decrease in complex I and IV activities can develop rapidly within 1 year. In Parkinson-plus patients suffering from multiple system atrophy the ETC activities were normal.
我们采用一种仅需100毫升血液的技术，对44名对照者、27名特发性帕金森病患者和8名因多系统萎缩导致的帕金森叠加综合征患者血小板线粒体中的电子传递复合体（ETC）I、III和IV进行了研究。在对照者和患者中，ETC测量均每隔几个月重复进行一次。正常受试者的活性差异很大，但重复测量时ETC活性的个体内差异较小。在正常受试者中，酶活性与年龄或训练状态之间无相关性。对照组中吸烟者和非吸烟者的酶活性无差异。帕金森病患者的复合体I活性低于对照组（分别为14和29纳摩尔/分钟/毫克血小板蛋白；P<0.001）。此外，复合体IV活性的组间差异也具有统计学意义（分别为83和58纳摩尔/分钟/毫克血小板蛋白；P<0.001）。此外，在一些帕金森病患者中，复合体III的活性较低，超出了对照范围，但组间差异未达到显著水平。帕金森病患者的复合体I活性与疾病持续时间、严重程度以及每日左旋多巴剂量之间无相关性。对5名处于疾病最早阶段的帕金森病患者进行重复测量表明，复合体I和IV活性的降低可在1年内迅速出现。在患有多系统萎缩的帕金森叠加综合征患者中，ETC活性正常。
