COX14
Description
The COX14 (cytochrome c oxidase assembly factor COX14) is a protein-coding gene located on chromosome 12.
Cytochrome c oxidase assembly factor COX14 is a protein that in humans is encoded by the COX14 gene. This gene encodes a small single-pass transmembrane protein that localizes to mitochondria. This protein may play a role in coordinating the early steps of cytochrome c oxidase (COX; also known as complex IV) subunit assembly and, in particular, the synthesis and assembly of the COX I subunit of the holoenzyme. Mutations in this gene have been associated with mitochondrial complex IV deficiency. Alternative splicing results in multiple transcript variants.
== Structure == The COX14 gene is located on the q arm of chromosome 12 at position 13.12 and it spans 8,476 base pairs. The COX14 gene produces a 6.6 kDa protein composed of 57 amino acids. COX14 is a component of the enzyme MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complex) complex, and the structure contains a central transmembrane domain.
== Function == The COX14 gene encodes for a core protein component of the MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase complex) complex, which is required for the proper regulation of complex IV assembly. Complex IV of the mitochondrial respiratory chain is essential in catalyzing the oxidation of cytochrome c by molecular oxygen.
COX14 is also known as C12orf62, MC4DN10, PCAG1.
