NDUFS3
NDUFS3 (NADH:ubiquinone oxidoreductase core subunit S3, also known as CI-30) is a core iron-sulfur protein subunit of mitochondrial complex I. It is essential for the catalytic activity and assembly of the NADH dehydrogenase complex of the respiratory chain. Browse Assay Genie's current range of NDUFS3 research reagents below, or read on for the biology behind the target.
NDUFS3 is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, also called complex I. Complex I catalyses electron transfer from NADH through the respiratory chain, using ubiquinone as the electron acceptor, and is assigned EC 7.1.1.2. According to UniProt, NDUFS3 is essential for both the catalytic activity and the assembly of complex I, so loss of the subunit has consequences for the whole enzyme complex rather than for a single peripheral function.
The NDUFS3 gene encodes one of the iron-sulfur protein (IP) components of mitochondrial NADH:ubiquinone oxidoreductase. The protein is localised to the mitochondrial inner membrane and belongs to the complex I 30 kDa subunit family. HGNC places it in the group of NADH:ubiquinone oxidoreductase core subunits. The mouse ortholog is Ndufs3, which is relevant when planning work in murine models.
The name reflects the older nomenclature, in which the gene was described as NADH dehydrogenase (ubiquinone) Fe-S protein 3 (30kD) (NADH-coenzyme Q reductase). The alias CI-30 refers to the same 30 kDa complex I subunit. Mutations in the NDUFS3 gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency, making it a useful reference point for studies of respiratory chain assembly and mitochondrial disease.
NDUFS3 is a core subunit of complex I, which transfers electrons from NADH to ubiquinone in the respiratory chain.
UniProt describes NDUFS3 as essential for complex I assembly, so it is a useful subunit for studying how the enzyme is built.
Mutations in NDUFS3 are associated with Leigh syndrome caused by mitochondrial complex I deficiency.
NDUFS3 is one of the iron-sulfur protein components of complex I, linking it to studies of respiratory chain composition.
NDUFS3 is a core subunit of mitochondrial complex I (NADH dehydrogenase), which catalyses electron transfer from NADH through the respiratory chain with ubiquinone as the electron acceptor. UniProt reports that it is essential for the catalytic activity and assembly of complex I.
NDUFS3 is also known as CI-30. Earlier names include NADH dehydrogenase (ubiquinone) Fe-S protein 3 (30kD) (NADH-coenzyme Q reductase). UniProt lists the protein as NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial.
Mutations in the NDUFS3 gene are associated with Leigh syndrome resulting from mitochondrial complex I deficiency. A mouse ortholog, Ndufs3, exists. Check the datasheet of any product for its validated reactivity.
Other NDUFS subunits of mitochondrial complex I and related respiratory chain targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt O75489; product specifications from Assay Genie datasheets.