Wijburg F A, Feller N, Scholte H R, Przyrembel H, Wanders R J
Department of Pediatrics, University Hospital, Amsterdam, The Netherlands.
Biochem Int. 1989 Sep;19(3):563-70.
We investigated the time course of the formation of lactate and pyruvate from glucose in cultured skin fibroblasts from controls, from a patient with a cytochrome c oxidase deficiency and from controls treated with inhibitors of the individual respiratory chain complexes. Fibroblasts from the patient and inhibitor treated fibroblasts produced more lactate and less pyruvate; this resulted in a significant increase in the lactate to pyruvate ratio, reflecting an increased cytosolic NADH/NAD+ redox state. We conclude that measurement of lactate and pyruvate production from glucose in cultured skin fibroblasts can be of value in the diagnosis of inherited diseases of the mitochondrial respiratory chain.
我们研究了来自对照组、一名细胞色素c氧化酶缺乏症患者以及用各个呼吸链复合物抑制剂处理的对照组的培养皮肤成纤维细胞中，葡萄糖生成乳酸和丙酮酸的时间进程。该患者的成纤维细胞以及经抑制剂处理的成纤维细胞产生了更多的乳酸和更少的丙酮酸；这导致乳酸与丙酮酸的比率显著增加，反映出胞质中NADH/NAD⁺氧化还原状态升高。我们得出结论，测量培养的皮肤成纤维细胞中葡萄糖生成乳酸和丙酮酸的情况，对于线粒体呼吸链遗传性疾病的诊断可能具有价值。
