NDUFS6
NDUFS6 (NADH:ubiquinone oxidoreductase subunit S6, also known as CI-13kA) is an accessory subunit of mitochondrial respiratory chain Complex I, the first enzyme complex of the electron transport chain. It is thought not to take part in catalysis. Browse Assay Genie's current range of NDUFS6 research reagents below, or read on for the biology behind the target.
NDUFS6 is an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, known as Complex I. Complex I transfers electrons from NADH to the respiratory chain, and the immediate electron acceptor for the enzyme is believed to be ubiquinone. NDUFS6 is believed not to be involved in catalysis itself, so its role is generally considered to be supporting the complex rather than driving electron transfer.
The human NDUFS6 gene encodes a protein that localises to the mitochondrial inner membrane. It belongs to the complex I NDUFS6 subunit family and sits in the HGNC gene group of NADH:ubiquinone oxidoreductase supernumerary subunits. According to the NCBI Gene summary, the encoded subunit is one of seven subunits in the iron-sulfur protein fraction of Complex I. Mouse and rat both carry an orthologous Ndufs6 gene, which allows the protein to be studied in common model systems.
The protein has also been called NADH dehydrogenase (ubiquinone) Fe-S protein 6 (13kD), or NADH-coenzyme Q reductase, which explains the older CI-13kA alias. Mutations in the NDUFS6 gene cause mitochondrial complex I deficiency, a disease that presents as a wide variety of clinical disorders, from neonatal disease to adult-onset neurodegenerative disorders. For this reason, NDUFS6 is a useful reference point for work on Complex I assembly, function and dysfunction.
NDUFS6 is a subunit of Complex I, the first enzyme complex of the electron transport chain, which transfers electrons from NADH to the respiratory chain.
Mutations in NDUFS6 cause mitochondrial complex I deficiency, making it a relevant gene for studying this disease and its clinical presentations.
Complex I deficiency linked to NDUFS6 mutations spans neonatal disease and adult-onset neurodegenerative disorders, supporting its use in neurological and mitochondrial studies.
As a supernumerary subunit within the iron-sulfur protein fraction, NDUFS6 helps researchers examine how accessory subunits contribute to the complex.
NDUFS6 is an accessory subunit of mitochondrial Complex I (NADH dehydrogenase). Complex I transfers electrons from NADH to the respiratory chain, with ubiquinone believed to be the immediate acceptor. NDUFS6 itself is believed not to be involved in catalysis. Check the datasheet of any product for its validated reactivity.
The official name is NADH:ubiquinone oxidoreductase subunit S6, and CI-13kA is a recorded alias. UniProt lists it as NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial. Earlier names included NADH dehydrogenase (ubiquinone) Fe-S protein 6 (13kD) (NADH-coenzyme Q reductase).
Yes. Mutations in the NDUFS6 gene cause mitochondrial complex I deficiency, which produces a wide variety of clinical disorders, including neonatal disease and adult-onset neurodegenerative disorders. Mouse and rat both have an orthologous gene, Ndufs6.
Other NDUFS subunits of mitochondrial Complex I and related electron transport chain targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt O75380; product specifications from Assay Genie datasheets.