title: A deficiency in respiratory complex I in heart mitochondria from vitamin A-deficient rats is counteracted by an increase in coenzyme Q.
abstract: Defects of NADH:coenzyme Q oxidoreductase (complex I) of mitochondria have been described in many congenital and acquired diseases. Administration of coenzyme Q (CoQ, ubiquinone) has been shown to benefit patients with some of these diseases. However, the mechanisms by which CoQ exerts the therapeutic effects are not clearly understood. A reason could be the lack of saturation of CoQ, in kinetic terms, for complex I activity. However, this hypothesis has not been proved in vivo because of the difficulty to incorporate CoQ into the mitochondrial membranes. We have found a deficiency in respiratory complex I in heart mitochondria from vitamin A-deficient rats which was accompanied by high CoQ content. The defect in complex I activity was compensated by the increase in CoQ to maintain the mitochondrial electron transfer rate. This finding supports, for the first time in an in vivo experimental approach, the kinetic hypothesis to explain the short-term therapeutic effects of CoQ.
authors.affiliation: Departament de Bioquímica i Biologia Molecular, Facultat de Farmàcia,Universitat de València, Burjassot, Spain. Ernesto.Estornell@uv.es
authors.forename: E
authors.initials: E
authors.lastname: Estornell
authors.forename: J R
authors.initials: JR
authors.lastname: Tormo
authors.forename: T
authors.initials: T
authors.lastname: Barber
mesh_terms: D000818:Animals; D042967:Electron Transport Complex I; D007700:Kinetics; D008929:Mitochondria, Heart / Q000201:enzymology*; D009247:NADH, NADPH Oxidoreductases / Q000378:metabolism*; D051381:Rats; D014451:Ubiquinone / Q000378:metabolism*; D014802:Vitamin A Deficiency / Q000201:enzymology*
chemical_list: D014451:Ubiquinone; D009247:NADH, NADPH Oxidoreductases; D042967:Electron Transport Complex I