Oxidative phosphorylation analysis

Assessing the integrated functional activity of human skeletal muscle mitochondria - Case studies

Michelle Puchowicz, Marie E. Varnes, Bruce H. Cohen, Neil R. Friedman, Douglas S. Kerr, Charles L. Hoppel

Research output: Contribution to journalArticle

51 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)377-385
Number of pages9
JournalMitochondrion
Volume4
Issue number5-6 SPEC. ISS.
DOIs
StatePublished - Jan 1 2004
Externally publishedYes

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Muscle Mitochondrion
Oxidative Phosphorylation
Human Activities
Mitochondria
Skeletal Muscle
Carnitine
Electron Transport
Fatty Acids
Fumarate Hydratase
Acetylcarnitine
Palmitoyl Coenzyme A
Pyruvate Dehydrogenase Complex
Ubiquinone
Adenine Nucleotides
Mitochondrial Membranes
Succinic Acid
Pyruvic Acid
Detergents
Glutamic Acid
Oxidoreductases

All Science Journal Classification (ASJC) codes

  • Molecular Medicine
  • Molecular Biology
  • Cell Biology

Cite this

Oxidative phosphorylation analysis : Assessing the integrated functional activity of human skeletal muscle mitochondria - Case studies. / Puchowicz, Michelle; Varnes, Marie E.; Cohen, Bruce H.; Friedman, Neil R.; Kerr, Douglas S.; Hoppel, Charles L.

In: Mitochondrion, Vol. 4, No. 5-6 SPEC. ISS., 01.01.2004, p. 377-385.

Research output: Contribution to journalArticle

Puchowicz, Michelle ; Varnes, Marie E. ; Cohen, Bruce H. ; Friedman, Neil R. ; Kerr, Douglas S. ; Hoppel, Charles L. / Oxidative phosphorylation analysis : Assessing the integrated functional activity of human skeletal muscle mitochondria - Case studies. In: Mitochondrion. 2004 ; Vol. 4, No. 5-6 SPEC. ISS. pp. 377-385.
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