Cytochrome c oxidase subunit 2

Cytochrome c oxidase subunit 2 (COX2), also known as cytochrome c oxidase polypeptide II, is an integral part of the mitochondrial respiratory chain that participates in cellular respiration. It is encoded by the mitochondrial gene MT-CO2.

Function

COX2 is a core subunit of the cytochrome c oxidase (COX) complex, also known as Complex IV, the last enzyme in the mitochondrial electron transport chain. It plays a crucial role in the production of adenosine triphosphate (ATP), the main source of cellular energy. COX2, along with other subunits, facilitates the transfer of electrons from cytochrome c to oxygen, a process that drives the synthesis of ATP.

Structure

COX2 is a transmembrane protein composed of 227 amino acids. It contains two heme a moieties and a copper center, which are essential for its enzymatic activity. The protein's structure allows it to function as a proton pump, contributing to the generation of a proton gradient across the mitochondrial inner membrane.

Clinical significance

Mutations in the MT-CO2 gene can lead to mitochondrial diseases such as Leber's hereditary optic neuropathy (LHON) and mitochondrial myopathy. These conditions are characterized by muscle weakness, vision loss, and other neurological problems.

File:Cytochrome c oxidase 2.png

Structure of cytochrome c oxidase subunit 2

See also

• Cytochrome c oxidase
• Mitochondrial diseases
• Leber's hereditary optic neuropathy
• Mitochondrial myopathy

References

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