Rabbit anti-Human NDUFS1 Polyclonal Antibody

Uniprot Id

P28331

Target Species

Human

Target Name

NDUFS1

Target Full Name

NADH-ubiquinone oxidoreductase 75 kDa subunit, 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 catalysing the entry and efficient transfer of electrons within complex I. Plays a key role in the assembly and stability of complex I and participates in the association of complex I with ubiquinol-cytochrome reductase complex (Complex III) to form supercomplexes.

Target Involvement

Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Complex I 75 kDa subunit family

Target Synonyms

CI-75kD; Complex I 75Kd; Complex I, mitochondrial respiratory chain, 75 kD subunit; Complex I-75kD; mitochondrial; NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) FeS protein 1 (75kD) (NADH coenzyme Q reductase); NADH ubiquinone oxidoreductase 75 kDa subunit mitochondrial ; NADH-ubiquinone oxidoreductase 75 kDa subunit; NDUFS1; NDUS1_HUMAN; PRO1304

Target Background

The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. 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. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency. Several transcript variants encoding different isoforms have been found for this gene.