Isoforms of Mammalian Cytochrome c Oxidase:Correlation with Human Cytochrome c Oxidase Deficiency
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چکیده
منابع مشابه
Assembly of cytochrome c oxidase: what can we learn from patients with cytochrome c oxidase deficiency?
Cytochrome c oxidase is an intricate metalloprotein that transfers electrons from cytochrome c to oxygen in the last step of the mitochondrial respiratory chain. It uses the free energy of this reaction to sustain a transmembrane electrochemical gradient of protons. Site-directed mutagenesis studies of bacterial terminal oxidases and the recent availability of refined crystal structures of the ...
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Mitochondria participate in a number of metabolic pathways, in the cell growth, aging process and cell death/ apoptosis but their principal biological function is energy production. More than 90% of ATP is produced by oxidative phosphorylation (OXPHOS) in the inner mitochondrial membrane. OXPHOS is composed of four multiprotein respiratory chain complexes, ATP synthase and two electron carriers...
متن کاملTissue- and Condition-Specific Isoforms of Mammalian Cytochrome c Oxidase Subunits: From Function to Human Disease
Cytochrome c oxidase (COX) is the terminal enzyme of the electron transport chain and catalyzes the transfer of electrons from cytochrome c to oxygen. COX consists of 14 subunits, three and eleven encoded, respectively, by the mitochondrial and nuclear DNA. Tissue- and condition-specific isoforms have only been reported for COX but not for the other oxidative phosphorylation complexes, suggesti...
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Within the past year, the structures of the cytochrome c oxidase from the soil bacterium Paracoccus denitrificans and of the metal centers of the cytochrome c oxidase from bovine heart mitochondria, both determined at 2.8 A resolution by X-ray crystallography, have been reported. The structures form a basis for understanding the mechanism of this redox-coupled transmembrane proton pump, which i...
متن کاملThe Cytochrome C-cytochrome Oxidase Complex
In the preceding paper the oxidation of substrates by “indophenol oxidase” was demonstrated to be a joint action of cytochrome and cytochrome oxidase. It was further shown that with a given amount of oxidase the velocity of hydroquinone oxidation reached a maximum as the amount of added cytochrome was increased. The latter fact immediately suggested the probability that the rapid aerobic oxidat...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1990
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199011000-00024