COX6A1 Antibody – Rabbit Polyclonal

Cytochrome c oxidase (COX), the terminal enzyme of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. It is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in the electron transfer and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes polypeptide 1 (liver isoform) of subunit VIa, and polypeptide 1 is found in all non-muscle tissues. Polypeptide 2 (heart/muscle isoform) of subunit VIa is encoded by a different gene, and is present only in striated muscles. These two polypeptides share 66% amino acid sequence identity. It has been reported that there may be several pseudogenes on chromosomes 1, 6, 7q21, 7q31-32 and 12. However, only one pseudogene (COX6A1P) on chromosome 1p31.1 has been documented. [provided by RefSeq, Jul 2008]

COX6A1: This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport. Belongs to the cytochrome c oxidase subunit 6A family.

Protein type: Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 12q24.2|12q24.2

Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex IV

Molecular Function: cytochrome-c oxidase activity

Biological Process: generation of precursor metabolites and energy; mitochondrial electron transport, cytochrome c to oxygen

Disease: Charcot-marie-tooth Disease, Recessive Intermediate D