Rabbit anti-Human NDUFS7 Polyclonal Antibody

Uniprot Id

O75251

Target Species

Human

Target Name

NDUFS7

Target Full Name

NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, 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.

Target Involvement

Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Complex I 20 kDa subunit family

Target Synonyms

CI 20; CI-20kD; Complex I 20kDa subunit; Complex I mitochondrial respiratory chain 20 KD subunit; Complex I-20kD; FLJ45860; FLJ46880; MGC120002; MY017; NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) Fe S protein 7 20kDa (NADH coenzyme Q reductase); NADH dehydrogenase (ubiquinone) FeS protein 7; 20kDa (NADHcoenzyme Q reductase) ; NADH dehydrogenase (ubiquinone) FeS protein7; 20kDa (NADHcoenzyme Q reductase); NADH dehydrogenase [ubiquinone] iron-sulfur protein 7; mitochondrial; NADH-ubiquinone oxidoreductase 20 kDa subunit; NADH:ubiquinone oxidoreductase PSST subunit ; NADHcoenzyme Q reductase; Ndufs7; NDUS7_HUMAN; PSST; PSST subunit

Target Background

This gene encodes a protein that is a subunit of one of the complexes that forms the mitochondrial respiratory chain. This protein is one of over 40 subunits found in complex I, the nicotinamide adenine dinucleotide (NADH):ubiquinone oxidoreductase. This complex functions in the transfer of electrons from NADH to the respiratory chain, and ubiquinone is believed to be the immediate electron acceptor for the enzyme. Mutations in this gene cause Leigh syndrome due to mitochondrial complex I deficiency, a severe neurological disorder that results in bilaterally symmetrical necrotic lesions in subcortical brain regions.