NDUFS4
NDUFS4 (NADH:ubiquinone oxidoreductase subunit S4, also known as AQDQ and CI-18) is a nuclear-encoded accessory subunit of mitochondrial respiratory chain Complex I. It sits in the inner mitochondrial membrane and is believed not to take part directly in catalysis. Browse Assay Genie's current range of NDUFS4 research reagents below, or read on for the biology behind the target.
NDUFS4 is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, also called Complex I or NADH:ubiquinone oxidoreductase. Complex I transfers electrons from NADH to the respiratory chain, and the immediate electron acceptor for the enzyme is believed to be ubiquinone. The NDUFS4 subunit is believed not to be involved in catalysis, so its role is thought to be supporting rather than directly enzymatic.
The NDUFS4 gene is nuclear-encoded, and the resulting NDUFS4 protein is targeted to the mitochondrion, where it is found at the inner membrane. The protein is a member of the complex I NDUFS4 subunit family. HGNC places it in the group of NADH:ubiquinone oxidoreductase supernumerary subunits. Alternative splicing of the gene results in multiple transcript variants. Mouse and rat both carry an orthologous gene, Ndufs4, which allows the protein to be studied in common rodent models.
The protein's earlier names reflect its apparent size and its function: NADH dehydrogenase (ubiquinone) Fe-S protein 4 (18kD) and NADH-coenzyme Q reductase. The aliases AQDQ and CI-18 are also in use. Mutations in NDUFS4 can cause mitochondrial complex I deficiencies such as Leigh syndrome, which makes the gene a frequent point of reference in work on respiratory chain disorders. Researchers studying NDUFS4 often look at it alongside other Complex I subunits, such as NDUFS1, NDUFS2 and NDUFS3.
Mutations in NDUFS4 can cause complex I deficiencies such as Leigh syndrome, making it a key gene in studies of mitochondrial disease.
NDUFS4 is an accessory subunit of Complex I, which moves electrons from NADH to the respiratory chain.
As a supernumerary subunit believed not to be involved in catalysis, NDUFS4 is useful for examining the non-catalytic parts of Complex I.
Mouse and rat both have an Ndufs4 ortholog, supporting comparative work on this Complex I subunit in model organisms.
NDUFS4 is an accessory subunit of mitochondrial Complex I, the NADH dehydrogenase of the respiratory chain. Complex I passes electrons from NADH to ubiquinone. NDUFS4 is believed not to be involved in catalysis itself. Check the datasheet of any product for its validated reactivity.
NDUFS4 is also known as AQDQ and CI-18. Its UniProt name is NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial. Earlier names include NADH dehydrogenase (ubiquinone) Fe-S protein 4 (18kD) and NADH-coenzyme Q reductase. Alternative splicing gives multiple transcript variants.
Yes. Mutations in the NDUFS4 gene can cause mitochondrial complex I deficiencies such as Leigh syndrome. Orthologous genes named Ndufs4 exist in both mouse and rat, so the protein can be studied in rodent systems.
Other NADH:ubiquinone oxidoreductase iron-sulfur subunits and Complex I components with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt O43181; product specifications from Assay Genie datasheets.