Puchowicz 2004 Mitochondrion

Puchowicz MA, Varnes ME, Cohen BH, Friedman NR, Kerr DS, Hoppel CL (2004) Oxidative phosphorylation analysis: assessing the integrated functional activity of human skeletal muscle mitochondria-case studies. Mitochondrion 4: 377-385.

» [[Has info::PMID:16120399]]

Puchowicz MA, Varnes ME, Cohen BH, Friedman NR, Kerr DS, Hoppel CL (2004) Mitochondrion

Abstract: 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. • Keywords: Oxidative phosphorylation; Skeletal muscle mitochondria; Adenine nucleotide translocator; Pyruvate dehydrogenase complex; Uncoupling; Carnitine palmitoyltransferase

• O2k-Network Lab: US_OH Cleveland_Hoppel CL

Labels:

Organism: Human  Tissue;cell: Skeletal muscle  Preparation: Isolated Mitochondria  Enzyme: TCA Cycle and Matrix Dehydrogenases, Inner mtMembrane Transporter  Regulation: Respiration; OXPHOS; ETS Capacity, Substrate; Glucose; TCA Cycle