Rabbit anti-Human NDUFA4 Polyclonal Antibody

Uniprot Id

O00483

Target Species

Human

Target Name

NDUFA4

Target Full Name

Cytochrome c oxidase subunit NDUFA4

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 unsing 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix. NDUFA4 is required for complex IV maintenance.

Target Involvement

Leigh syndrome (LS)

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein.

Target Protein Families

Complex IV NDUFA4 subunit family

Target Research Area

Metabolism

Target Synonyms

CI 9k; CI-MLRQ; Complex I 9kDa subunit; Complex I-MLRQ; MLRQ; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 4 9kD; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4; NADH-ubiquinone oxidoreductase MLRQ subunit; NDUA4_HUMAN; NDUFA4

Target Background

The protein encoded by this gene belongs to the complex I 9kDa subunit family. Mammalian complex I of mitochondrial respiratory chain is composed of 45 different subunits. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.