Superoxide production is inversely related to complex I activity in inherited complex I deficiency

• Published in: Biochimica et biophysica acta Vol. 1772; no. 3; pp. 373 - 381
• Main Authors: Verkaart, Sjoerd, Koopman, Werner J.H., van Emst-de Vries, Sjenet E., Nijtmans, Leo G.J., van den Heuvel, Lambertus W.P.J., Smeitink, Jan A.M., Willems, Peter H.G.M.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.03.2007; Elsevier
• Subjects: Child, Preschool; Electron Transport Complex I - analysis; Electron Transport Complex I - deficiency; Electron Transport Complex I - genetics; Female; Fibroblast; Fibroblasts - enzymology; Fluorescence microscopy; Humans; Hydroethidine; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors - enzymology; Metabolism, Inborn Errors - genetics; Metabolism, Inborn Errors - metabolism; Mitochondria; Oxidative Phosphorylation; Rotenone; Rotenone - administration & dosage; Skin - enzymology; Superoxides - analysis; Superoxides - metabolism; Uncoupling Agents - administration & dosage; CIV; Δ ψ; CII; Mitochondria; Hydroethidine; CI; HEt; CIII; OXPHOS; Fibroblast; Fluorescence microscopy; Rotenone; Life Sciences
• ISSN: 0925-4439; 0006-3002; 1879-260X
• DOI: 10.1016/j.bbadis.2006.12.009

Deficiency of NADH:ubiquinone oxidoreductase or complex I (CI) is the most common cause of disorders of the oxidative phosphorylation system in humans. Using life cell imaging and blue-native electrophoresis we quantitatively compared superoxide production and CI amount and activity in cultured skin...