COX IV(Cytochrome c oxidase) is a terminal enzyme complex involved in the mitochondrial electron transport chain. Consisting of 13 subunits, it is localized at the inner mitochondrial membrane. One of the small subunits of COX IV is encoded by nuclear DNA while the 3 largest subunits forming the catalytic core are encoded by mitochondrial DNA. Deficiency in COX activity leads to a number of human diseases. The COX IV antibody can be used effectively as a mitochondrial loading control.