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Detection of respiratory chain dysfunction by measuring lactate and pyruvate production in cultured fibroblasts.

作者信息

Wijburg F A, Feller N, Ruitenbeek W, Trijbels J M, Sengers R C, Scholte H R, Przyrembel H, Wanders R J

机构信息

Department of Pediatrics, University Hospital of Amsterdam (AMC), The Netherlands.

出版信息

J Inherit Metab Dis. 1990;13(3):355-8. doi: 10.1007/BF01799394.

DOI:10.1007/BF01799394

PMID:2172645

Abstract

摘要

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Oxidative phosphorylation in mitochondria isolated from human fibroblasts.

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A mitochondrial encephalomyopathy: the first case with an established defect at the level of coenzyme Q.

Eur J Pediatr. 1986 Feb;144(5):441-4. doi: 10.1007/BF00441735.

Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.

J Pediatr. 1987 Feb;110(2):216-22. doi: 10.1016/s0022-3476(87)80157-9.

Respiratory chain defects in the mitochondria of cultured skin fibroblasts from three patients with lacticacidemia.

J Clin Invest. 1986 May;77(5):1422-7. doi: 10.1172/JCI112453.

Isozymes of cytochrome-c oxidase: characterization and isolation from different tissues.

Methods Enzymol. 1986;126:32-45. doi: 10.1016/s0076-6879(86)26006-1.

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Detection of respiratory chain dysfunction by measuring lactate and pyruvate production in cultured fibroblasts.

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