Wijburg F A, Feller N, de Groot C J, Wanders R J
Department of Pediatrics, University Hospital of Amsterdam (AMC), The Netherlands.
Biochem Int. 1990 Oct;22(2):303-9.
In this paper we report our studies on the effects of menadione in cultured fibroblasts treated with rotenone to block complex I. A normalization of the lactate to pyruvate ratio after incubation with glucose, an increased production of 14CO2 from [6-14C]glucose and an increased intra-cellular concentration of ATP was observed in the presence of micromolar concentrations of menadione. These results not only demonstrate the potential value of menadione in complex I deficient patients but also suggest that this system can be used advantageously for the in vitro assessment of therapeutic agents for disorders of the mitochondrial respiratory chain.
在本文中，我们报告了我们对甲萘醌在经鱼藤酮处理以阻断复合体I的培养成纤维细胞中的作用的研究。在存在微摩尔浓度甲萘醌的情况下，观察到与葡萄糖孵育后乳酸与丙酮酸比值的正常化、[6-¹⁴C]葡萄糖产生¹⁴CO₂的增加以及细胞内ATP浓度的增加。这些结果不仅证明了甲萘醌在复合体I缺陷患者中的潜在价值，还表明该系统可有利地用于体外评估线粒体呼吸链疾病的治疗药物。
