mitochondrial respiratory chain complex IV

Definition

Target type: cellularcomponent

A protein complex located in the mitochondrial inner membrane that forms part of the mitochondrial respiratory chain. Contains the 13 polypeptide subunits of cytochrome c oxidase, including cytochrome a and cytochrome a3. Catalyzes the oxidation of reduced cytochrome c by dioxygen (O2). [GOC:mtg_sensu, ISBN:0198547684]

Mitochondrial respiratory chain complex IV, also known as cytochrome c oxidase, is a large transmembrane protein complex located in the inner mitochondrial membrane. It is the terminal enzyme in the electron transport chain, responsible for the reduction of molecular oxygen to water, coupling this reaction to the pumping of protons across the inner mitochondrial membrane, thus generating a proton gradient that drives ATP synthesis. The complex consists of 13 subunits in mammals, including three core subunits (COX1, COX2, COX3) encoded by mitochondrial DNA and 10 nuclear-encoded subunits. The core subunits contain the heme and copper centers responsible for electron transfer, while the nuclear-encoded subunits contribute to assembly, stability, and regulation of the complex. Complex IV is a highly conserved protein complex, with homologs found in all aerobic organisms. It is essential for cellular respiration and ATP production, and defects in its function can lead to a variety of human diseases, including mitochondrial myopathy and Leigh syndrome.'
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Proteins (4)

Compounds (9)