Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency.
until further notice
SourceInternational Journal of Biochemistry & Cell Biology, 41, 10, (2009), pp. 1773-82
Article / Letter to editor
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Cell Biology (UMC)
Paediatrics - OUD tm 2017
International Journal of Biochemistry & Cell Biology
SubjectIGMD 8: Mitochondrial medicine; NCMLS 4: Energy and redox metabolism
Mitochondrial NADH:ubiquinone oxidoreductase or complex I (CI) is a frequently affected enzyme in cases of mitochondrial disorders. However, the cytopathological mechanism of the associated pediatric syndromes is poorly understood. Evidence in the literature suggests a connection between mitochondrial metabolism and morphology. Previous quantitative analysis of mitochondrial structure in cultured fibroblasts of 14 patients revealed that mitochondria were fragmented and/or less branched in patients with severe CI deficiency. These patient cells also displayed greatly increased levels of reactive oxygen species (ROS) and marked aberrations in mitochondrial and cellular Ca(2+)/ATP handling upon hormone stimulation. Here, we discuss the interrelationship between these parameters and demonstrate that the hormone-induced increase in mitochondrial Ca(2+) and ATP concentration, as well as the rate of cytosolic Ca(2+) removal, are not related to mitochondrial length and/or degree of branching, but decrease as a function of the number of mitochondria per cell. This suggests that the amount of mitochondria, and not their shape, is important for Ca(2+)-induced stimulation of mitochondrial ATP generation to feed cytosolic ATP-demanding processes.
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