Cytochrome c oxidase subunit III

Cytochrome c oxidase subunit III (COX3) is a protein that in humans is encoded by the MT-CO3 gene. It is a component of the electron transport chain in mitochondria, which plays a crucial role in cellular respiration.

Function

Cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial respiratory chain. It plays an essential role in the transfer of electrons from cytochrome c to oxygen. This process drives the synthesis of adenosine triphosphate (ATP), the main source of energy in cells. COX3 is one of the three mitochondrial DNA (mtDNA)-encoded subunits of COX.

Structure

The MT-CO3 gene provides instructions for making a protein that is a subunit of the cytochrome c oxidase complex. This complex is a part of the mitochondrial respiratory chain and consists of 13 protein subunits. The MT-CO3 gene is located in mitochondrial DNA, which is found in the mitochondria.

Clinical significance

Mutations in the MT-CO3 gene have been associated with a variety of mitochondrial disorders. These include Leber's hereditary optic neuropathy (LHON), mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), and myoclonic epilepsy with ragged-red fibers (MERRF).

See also

• Cytochrome c oxidase
• Mitochondrial DNA
• Electron transport chain
• Cellular respiration

References

‎ ‎

---

Translate: - East Asian 中文, 日本, 한국어, South Asian हिन्दी, தமிழ், తెలుగు, Urdu, ಕನ್ನಡ, Southeast Asian Indonesian, Vietnamese, Thai, မြန်မာဘာသာ, বাংলা
European español, Deutsch, français, Greek, português do Brasil, polski, română, русский, Nederlands, norsk, svenska, suomi, Italian
Middle Eastern & African عربى, Turkish, Persian, Hebrew, Afrikaans, isiZulu, Kiswahili,
Other Bulgarian, Hungarian, Czech, Swedish, മലയാളം, मराठी, ਪੰਜਾਬੀ, ગુજરાતી, Portuguese, Ukrainian