PMID: 8581354Apr 1, 1995Paper

Oxygraphic evaluation of mitochondrial function in digitonin-permeabilized mononuclear cells and cultured skin fibroblasts of patients with chronic progressive external ophthalmoplegia

Biochemical and Molecular Medicine
D KunzC W Wallesch

Abstract

For quantitative elucidation of maximal mitochondrial oxidation capacities in human mononuclear cells, cultured human skin fibroblasts and human thrombocytes the optimal amount of digitonin for plasma membrane permeabilization was determined to be 5, 10, and 0.1 micrograms/10(6) cells, respectively. Using these concentrations the rate of respiration of permeabilized cells with the mitochondrial substrates succinate (+ rotenone) or glutamate + malate can be stimulated between two- and fourfold by ADP and inhibited by carboxyatractyloside. The maximal respiratory activities of well-characterized preparations of permeabilized mononuclear cells of five patients with chronic progressive external ophthalmoplegia were compared to healthy controls and a 30 to 50% decrease of the ADP-stimulated respiration rates with glutamate + malate and succinate + rotenone was detected. This is an indication for the presence of the mitochondrial defect in respiratory active blood cells. Additionally, for two of these patients the mitochondrial defects were proven to be detectable by the determination of maximal oxygen consumption rates of digitonin-permeabilized cultured skin fibroblasts. Therefore, the determination of maximal oxidation capacities ...Continue Reading

Citations

May 14, 2004·Journal of the Neurological Sciences·Kirstin Winkler-StuckWolfram S Kunz
Apr 3, 2004·Molecular Genetics and Metabolism·Barbara J MarriageD Moira Glerum
Feb 11, 2003·Analytical Biochemistry·Barbara J MarriageD Moira Glerum
Nov 26, 2010·Critical Care : the Official Journal of the Critical Care Forum·Fredrik SjövallEskil Elmér
Oct 19, 2007·The Journal of Investigative Dermatology·Annette S VincentKim P Wong
Sep 6, 2003·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Marilena GrecoGiuseppe Attardi
Dec 15, 2018·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Jarosław WalczakJoanna Szczepanowska
Jun 9, 1998·Annals of the New York Academy of Sciences·E S Avgoustiniatos, C K Colton

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