Cytochrome c oxidase assembly factor COX14 (COX14)

Cytochrome c oxidase assembly factor COX14 is a small, single-pass transmembrane protein localized in the inner mitochondrial membrane. It is an essential component of the MITRAC complex, which coordinates synthesis and early assembly of the mitochondrial-encoded COX1 subunit into cytochrome c oxidase (complex IV), the terminal enzyme of the electron transport chain. COX14 regulates negative feedback for translation of COX1, protects newly synthesized COX1 from turnover, and supports the assembly of a functional enzyme complex. Mutations in COX14 disrupt complex IV activity, leading to mitochondrial complex IV deficiency, a clinically heterogeneous disorder with neurological, muscular, and hepatic manifestations[1][2][3]. Key details: - COX14 is not a classical therapeutic target such as a receptor, enzyme, transporter, or ion channel. - No known drugs are currently reported to act directly on COX14. - Pathogenic variants result in severe mitochondrial diseases by impairing the assembly and function of complex IV of the mitochondrial electron transport chain[1][3].

Other names

C12orf62PCAG1MC4DN10MGC14288Cytochrome c oxidase assembly protein COX14Cytochrome c oxidase assembly homolog 14