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Decreased expression o ubiquinol-cytochrome c reductase subunits in patients exhibiting mitochondrial myopathy with progressive exercise intolerance |
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| Authors: | Mohamed Fouad Bouzidi, Hermann Sch gger, Jean-Marc Collombet, Henri Carrier, Fr d ric Flocard, Simone Quard, B n dicte Mousson,Catherine Godinot |
| Affiliation: | a Pathologies Mitochondriales - UMR 106, CNRS - UCB, Lyon I, France b Klinikum der Johann Wolfgang Goethe-Universität, Frankfurt am Main, Germany c Hopital Debrousse, Centre d'étude des Maladies Métaboliques, Lyon, France d Pathologie Neuromusculaire - CNRS, EP18, Faculté de Médecine A. Carrel, Lyon, France e Hôpital d'Instruction des Armées, Desgenettes, Lyon, France f Hôpital Cardiologique, Lyon, France |
| Abstract: | The expression of mitochondrial proteins of two patients suffering from myopathy with progressive exercise intolerance and exhibiting a deficiency in the enzymatic activity of complex III (ubiquinol-cytochrome c reductase) has been analyzed by immunological titration. In both patients, the Fe-S protein, the cytochrome b and the 9.5 kDa protein were decreased while the expression of the other complex III subunits were close to normal values. This data indicates that, in some mitochondrial myopathies, proteins of the respiratory chain complexes can be accumulated in mitochondria without being integrated into a functional complex. This may be explained either by a lack of control of the coordination between the synthesis of subunits of mitochondrial and nuclear origin or by a difference in the degradation rate of the various subunits which are not properly assembled. |
| Keywords: | Mitochondrial myopathy, ubiquinol - cytochrome c reductase, bc1 complex, complex III, mitochondrial biogenesis, exercise intolerance, cytochrome b mitochondrial DNA |
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