期刊
MITOCHONDRION
卷 4, 期 5-6, 页码 377-385出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2004.07.004
关键词
oxidative phosphorylation; skeletal muscle mitochondria; adenine nucleotide translocator; pyruvate dehydrogenase complex; uncoupling; carnitine palmitoyltransferase
Oxidative phosphorylation analysis, performed on freshly-isolated mitochondria, assesses the integrated function of the electron transport chain (ETC) coupled to ATP synthesis, membrane transport, dehydrogenase activities, and the structural integrity of the mitochondria. In this review, a case study approach is employed to highlight detection of defects in the adenine nucleotide translocator, the pyruvate dehydrogenase complex, fumarase, coenzyme Q function, fatty acid metabolism, and mitochondrial membrane integrity. Our approach uses the substrates glutamate, pyruvate, 2-ketoglutarate (coupled with malonate), malate, and fatty acid substrates (palmitoylcarnitine, octanoylcarnitine, palmitoyl-CoA (with carnitine), octanoyl-CoA (with carnitine), octanoate and acetylcarnitine) in addition to succinate, durohydroquinone and TMPD/ascorbate to uncover metabolic defects that would not be apparent from ETC assays performed on detergent-solubilized mitochondria. (C) 2004 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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