Rabbit anti-Human COX6B1 Polyclonal Antibody

Uniprot Id

P14854

Target Species

Human

Target Name

COX6B1

Target Full Name

Cytochrome c oxidase subunit 6B1

Target Function

Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.

Target Involvement

Mitochondrial complex IV deficiency (MT-C4D)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.

Target Protein Families

Cytochrome c oxidase subunit 6B family

Target Synonyms

COX 6B; COX VIb 1; COX VIb-1; COX6B; COX6B1; COXG; COXVIb1; CX6B1_HUMAN; Cytochrome c oxidase subunit 6B1; Cytochrome c oxidase subunit VIb; Cytochrome c oxidase subunit VIb isoform 1; cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous); Cytochrome c oxidase subunit Vib polypeptide 1

Target Background

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 electron transfer, and the nuclear-encoded subunits may be involved in the regulation and assembly of the complex. This nuclear gene encodes subunit VIb. Mutations in this gene are associated with severe infantile encephalomyopathy. Three pseudogenes COX6BP-1, COX6BP-2 and COX6BP-3 have been found on chromosomes 7, 17 and 22q13.1-13.2, respectively.