Cytochrome c oxidase subunit I (None)

Cytochrome c oxidase subunit 1 (COX1, also known as MT-CO1) is a core component of cytochrome c oxidase (complex IV), the terminal enzyme of the mitochondrial electron transport chain. This subunit is encoded by mitochondrial DNA and is highly conserved across eukaryotes and many prokaryotes. COX1 forms part of the catalytic core of cytochrome c oxidase, which catalyzes the transfer of electrons from cytochrome c to molecular oxygen, reducing it to water. This process is coupled with proton pumping across the inner mitochondrial membrane, contributing to the generation of a proton gradient that drives ATP synthesis via oxidative phosphorylation. Defects or mutations in MT-CO1 can cause deficiencies in complex IV activity leading to various mitochondrial diseases with heterogeneous clinical presentations—ranging from muscle weakness to severe multi-system disorders depending on mutation severity/location.

Other names

COIMT-COICoxI

Inhibiting electron transport and ATP production