Rabbit anti-Human NDUFS2 Polyclonal Antibody

Uniprot Id

O75306

Target Species

Human

Target Name

NDUFS2

Target Full Name

NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial

Target Function

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for the catalytic activity of complex I. Essential for the assembly of complex I. Redox-sensitive, critical component of the oxygen-sensing pathway in the pulmonary vasculature which plays a key role in acute pulmonary oxygen-sensing and hypoxic pulmonary vasoconstriction. Plays an important role in carotid body sensing of hypoxia. Essential for glia-like neural stem and progenitor cell proliferation, differentiation and subsequent oligodendrocyte or neuronal maturation.

Target Involvement

Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Complex I 49 kDa subunit family

Target Synonyms

CI 49; CI 49kD ; CI-49kD; Complex 1, mitochondrial respiratory chain, 49 KD subunit; Complex I 49kD; Complex I 49kDa subunit; Complex I-49kD; mitochondrial; NADH dehydrogenase (ubiquinone) Fe S protein 2 49kDa; NADH dehydrogenase (ubiquinone) Fe S protein 2, 49kDa (NADH coenzyme Q reductase); NADH dehydrogenase [ubiquinone] iron sulfur protein 2, mitochondrial ; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2; NADH ubiquinone oxidoreductase 49 kDa subunit; NADH ubiquinone oxidoreductase NDUFS2 subunit; NADH-ubiquinone oxidoreductase 49 kDa subunit; NADH:ubiquinone oxidoreductase core subunit S2; Ndufs2; NDUS2_HUMAN

Target Background

The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.