Cytochrome c oxidase subunit 8A (COX8A) is a protein that in humans is encoded by the COX8Agene.[1] Cytochrome c oxidase 8A is a subunit of the cytochrome c oxidase complex, also known as Complex IV. Mutations in the COX8A gene have been associated with complex IV deficiency with Leigh syndrome and epilepsy.[2]
COX8A is a 7.6 kDa protein composed of 69 amino acids.[3][4] This gene encodes the nuclear-encoded subunit 8A of the human mitochondrialrespiratory chain enzyme complex cytochrome c oxidase. The complex consists of 13 mitochondrial- and nuclear-encoded subunits.[1]
Function
Cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrialrespiratory chain. It is a multi-subunit enzyme complex that couples the transfer of electrons from cytochrome c to molecular oxygen and contributes to a proton electrochemical gradient across the inner mitochondrial membrane. The mitochondrially-encoded subunits perform the electron transfer of proton pumping activities. The functions of the nuclear-encoded subunits are unknown but they may play a role in the regulation and assembly of the complex.[1]