Mitochondrial abnormalities in fibroblast line GM3093 defective in oxidative metabolism.
Fibroblast line GM3093 deficient in the activity of the pyruvate dehydrogenase complex, was derived from a patient reported to have an inherited defect affecting the tricarboxylic acid cycle. Our results suggest a generalized defect consisting of few and abnormal mitochondria and low activities of all mitochondrial enzymes examined.
Constantopoulos
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G
Greenwood
M A
MA
Sorrell
S H
SH
D002460:Cell Line; D002952:Citric Acid Cycle; D008058:Dihydrolipoamide Dehydrogenase / Q000378:metabolism; D005347:Fibroblasts / Q000648:ultrastructure*; D005969:Glutamate Dehydrogenase / Q000378:metabolism; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000378:metabolism; D007655:Ketoglutarate Dehydrogenase Complex / Q000378:metabolism; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D008854:Microscopy, Electron; D008928:Mitochondria / Q000201:enzymology / Q000648:ultrastructure*; D015325:Pyruvate Dehydrogenase Complex Deficiency Disease
D007770:L-Lactate Dehydrogenase; D007521:Isocitrate Dehydrogenase; D007655:Ketoglutarate Dehydrogenase Complex; D005969:Glutamate Dehydrogenase; D008058:Dihydrolipoamide Dehydrogenase