title: Decreased expression of ubiquinol-cytochrome c reductase subunits in patients exhibiting mitochondrial myopathy with progressive exercise intolerance.
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.
authors.affiliation: UMR 106, CNRS-UCB Lyon I, France.
authors.forename: M F
authors.initials: MF
authors.lastname: Bouzidi
authors.forename: H
authors.initials: H
authors.lastname: Schägger
authors.forename: J M
authors.initials: JM
authors.lastname: Collombet
authors.forename: H
authors.initials: H
authors.lastname: Carrier
authors.forename: F
authors.initials: F
authors.lastname: Flocard
authors.forename: S
authors.initials: S
authors.lastname: Quard
authors.forename: B
authors.initials: B
authors.lastname: Mousson
authors.forename: C
authors.initials: C
authors.lastname: Godinot
mesh_terms: D000328:Adult; D017668:Age of Onset; D000906:Antibodies; D014450:Electron Transport Complex III / Q000032:analysis / Q000096:biosynthesis* / Q000302:isolation & purification; D004591:Electrophoresis, Polyacrylamide Gel; D015444:Exercise; D006801:Humans; D046911:Macromolecular Substances; D008297:Male; D008931:Mitochondria, Muscle / Q000378:metabolism*; D017240:Mitochondrial Myopathies / Q000201:enzymology* / Q000235:genetics / Q000503:physiopathology*; D012016:Reference Values
chemical_list: D000906:Antibodies; D046911:Macromolecular Substances; D014450:Electron Transport Complex III