Mitochondrial cytochrome c oxidase deficiency
نویسندگان
چکیده
منابع مشابه
Reversible mitochondrial myopathy with cytochrome c oxidase deficiency.
Two siblings, a boy and a girl born in a nonconsanguineous marriage, presented with a similar clinical course. Sucking and breathing difficulties appeared within a few weeks of birth. Clinical examination revealed profound muscular hypotonia, hepatomegaly, increased serum creatine kinase activities, and lactic acidosis. Both infants were treated with gavage feeding, the boy also needing ventila...
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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...
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992 Introduction Cytochrome c oxidase is a fascinating enzyme that has attracted the attention of numerous researchers over the past two decades [ 1-61. The enzyme catalyses the one-electron oxidation of cytochrome c and the four-electron reduction of molecular oxygen to water. In doing so, the enzyme electrogenically pumps protons across the inner mitochondrial membrane, thus generating a tran...
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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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ژورنال
عنوان ژورنال: Clinical Science
سال: 2016
ISSN: 0143-5221,1470-8736
DOI: 10.1042/cs20150707