Mitochondrial abnormalities in fibroblast line GM3093 defective in oxidative metabolism |
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| Authors: | G Constantopoulos M A Greenwood S H Sorrell |
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| Institution: | (1) Developmental and Metabolic Neurology Branch and Surgical Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bldg 10, Rm 3D04, 20892 Bethesda, Maryland, USA |
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| Abstract: | Summary 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.Preliminary reports of aspects of this work appeared as abstracts in Trans. Am. Soc. Neurochem.15 (1984) 178, and Soc. Neurosci.10 (1984) 996.Developmental and Metabolic Neurology Branch.Surgical Neurology Branch. |
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| Keywords: | Mitochondria mitochondrial abnormalities mitochondrial enzymes oxidative metabolism fibroblast line GM3093 |
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