Recombinant Human NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (NDUFS3)

Uniprot Id

O75489

Target Species

Human

Target Name

NDUFS3

Target Full Name

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, 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 and assembly of complex I.

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Complex I 30 kDa subunit family

Target Synonyms

CI 30; CI 30KD; CI-30kD; Complex I 30KD; Complex I 30kDa subunit; COMPLEX I, MITOCHONDRIAL RESPIRATORY CHAIN, 30-KD SUBUNIT; Complex I-30kD; mitochondrial; NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) Fe S protein 3 30kDa; NADH dehydrogenase [ubiquinone] iron sulfur protein 3 mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3; NADH dehydrogenase ubiquinone 30 kDa subunit ; NADH-ubiquinone oxidoreductase 30 kDa subunit; NADH-Ubiquinone Oxidoreductase Fe-S Protein 3; NDUFS3; NDUS3_HUMAN

Target Background

This gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase (complex I). Mutations in this gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency.