NDUFV1
NDUFV1 (NADH:ubiquinone oxidoreductase core subunit V1, also known as CI-51K) is a core subunit of mitochondrial respiratory chain Complex I. It carries the NADH-binding site and the flavin mononucleotide (FMN) that accepts electrons from NADH. Browse Assay Genie's current range of NDUFV1 research reagents below, or read on for the biology behind the target.
NDUFV1 is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase, known as Complex I. This enzyme catalyses electron transfer from NADH through the respiratory chain, using ubiquinone as the electron acceptor. NDUFV1 sits in the peripheral arm of the enzyme. There, electrons from NADH are accepted by FMN and then passed along a chain of iron-sulfur clusters by electron tunnelling to ubiquinone. The protein contains the FMN, which is the initial electron acceptor, and one iron-sulfur cluster.
The NDUFV1 gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex. This large assembly has at least 45 nuclear and mitochondrial encoded subunits, and it liberates electrons from NADH and channels them to ubiquinone. The subunit carries the NADH-binding site as well as FMN- and Fe-S-binding sites. The protein is located at the mitochondrial inner membrane and belongs to the complex I 51 kDa subunit family. HGNC places it in the NADH:ubiquinone oxidoreductase core subunits and flavoprotein gene groups. Alternative splicing produces multiple transcript variants that encode distinct isoforms. The mouse ortholog is Ndufv1.
Complex I is one of four membrane-bound electron-transporting complexes (I-IV) that, with the ATP synthase (complex V), provide cellular energy through oxidative phosphorylation. Defects in complex I are a common cause of mitochondrial dysfunction, a syndrome occurring in roughly 1 in 10,000 live births. Complex I deficiency is linked to myopathies, encephalomyopathies and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. The earlier gene name, NADH dehydrogenase (ubiquinone) flavoprotein 1 (51kD), reflects the subunit's size.
NDUFV1 forms the NADH-binding and FMN-containing entry point of Complex I, making it a useful subunit for studying electron transfer to ubiquinone.
As a core subunit of Complex I, NDUFV1 supports research on how the respiratory chain and ATP synthase provide cellular energy.
Defects in complex I are a common cause of mitochondrial dysfunction, so NDUFV1 is relevant to work on myopathies and encephalomyopathies.
Complex I deficiency is linked to Parkinson's disease and Leigh syndrome, placing NDUFV1 within studies of neurodegenerative mitochondrial disease.
NDUFV1 is a core subunit of Complex I in the mitochondrial respiratory chain. In the peripheral arm, its FMN accepts electrons from NADH. These then pass along iron-sulfur clusters by electron tunnelling to ubiquinone. The subunit carries the NADH-binding site as well as FMN- and Fe-S-binding sites.
NDUFV1 is also called CI-51K, and UniProt names it NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial. Earlier names include NADH dehydrogenase (ubiquinone) flavoprotein 1 (51kD). Alternative splicing of the gene results in multiple transcript variants that encode distinct isoforms.
NDUFV1 is part of Complex I, and Complex I deficiency is linked to myopathies, encephalomyopathies, Parkinson's disease and Leigh syndrome. A mouse ortholog, Ndufv1, is recognised; check the datasheet of any product for its validated reactivity.
Other Complex I subunits and mitochondrial respiratory chain targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P49821; product specifications from Assay Genie datasheets.