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caaC Gene Product

The terminal oxidase of the respiratory chain. It collects electrons that are transferred from reduced CYTOCHROME C and donates them to molecular oxygen, which is then reduced to water. It is composed of CYTOCHROME A and CYTOCHROME B, two copper atoms, and 13 different protein subunits, three of which are encoded by the mitochondrial DNA. It is also called complex IV of the respiratory chain. (from Scriver et al., The Metabolic & Molecular Bases of Inherited Disease, 8th ed, p2261 and p2368)

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