NDUFV2

NDUFV2

Target · Mitochondrial enzyme subunit

NDUFV2 NADH:ubiquinone oxidoreductase core subunit V2 · NADH dehydrogenase [ubiquinone] flavoprotein 2

NDUFV2 (NADH:ubiquinone oxidoreductase core subunit V2, also known as CI-24k) is a core subunit of mitochondrial respiratory Complex I. It sits in the peripheral arm of the enzyme and takes part in electron transfer from NADH to ubiquinone. Browse Assay Genie's current range of NDUFV2 research reagents below, or read on for the biology behind the target.

Mitochondrial inner membraneComplex I core subunitLinked to neurological disease
249 aa
Protein length
~27 kDa
Molecular weight
18p11.22
Gene locus
EC 7.1.1.2
Enzyme class
Target information

What is NDUFV2 (NADH:ubiquinone oxidoreductase core subunit V2)?

NDUFV2 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, with ubiquinone as the electron acceptor. NDUFV2 belongs to the peripheral arm of the complex, where electrons from NADH are accepted by flavin mononucleotide (FMN) and then passed along a chain of iron-sulfur clusters by electron tunnelling to ubiquinone. The NDUFV2 protein contains one iron-sulfur cluster.

The NDUFV2 gene encodes the 24 kDa subunit of Complex I, which is why the protein is also called CI-24k. It belongs to the complex I 24 kDa subunit family and is classed by HGNC in the NADH:ubiquinone oxidoreductase core subunits group. Complex I is located in the inner mitochondrial membrane and consists of at least 43 subunits. Mouse and rat both have an orthologous gene, Ndufv2. A non-transcribed pseudogene of this locus is found on chromosome 19.

Earlier gene names described the product as NADH dehydrogenase (ubiquinone) flavoprotein 2 (24kD). Mutations in NDUFV2 are implicated in Parkinson's disease, bipolar disorder and schizophrenia, and have been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. This makes NDUFV2 a useful subject for studies of mitochondrial function and of disorders linked to Complex I.

Mitochondrial respiration

NDUFV2 is a core Complex I subunit that supports electron transfer from NADH to ubiquinone in the respiratory chain.

Complex I structure and assembly

As a peripheral arm subunit carrying an iron-sulfur cluster, NDUFV2 is relevant to how FMN and iron-sulfur centres pass electrons along the enzyme.

Neurological disease research

Mutations in NDUFV2 are implicated in Parkinson's disease, bipolar disorder and schizophrenia, linking Complex I function to neurological conditions.

Cardiac and metabolic disorders

NDUFV2 mutation has been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy, pointing to roles in heart and brain.

Background

NDUFV2 frequently asked questions

What does NDUFV2 do?

NDUFV2 is a core subunit of Complex I, the mitochondrial NADH dehydrogenase. In the peripheral arm, electrons from NADH are accepted by FMN and passed along iron-sulfur clusters to ubiquinone. NDUFV2 contributes one iron-sulfur cluster to this pathway and is located in the inner mitochondrial membrane.

What other names does NDUFV2 have?

NDUFV2 is also called CI-24k, after the 24 kDa subunit of Complex I. UniProt names the protein NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial. Earlier HGNC names were NADH dehydrogenase (ubiquinone) flavoprotein 2 (24kD) and the 24kDa variant. Check the datasheet of any product for its validated reactivity.

Is NDUFV2 linked to disease?

According to the NCBI Gene summary, NDUFV2 mutations are implicated in Parkinson's disease, bipolar disorder and schizophrenia. They have also been found in one case of early onset hypertrophic cardiomyopathy and encephalopathy. Mouse and rat carry the orthologous gene Ndufv2.

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Related targets

Other Complex I core subunits and mitochondrial respiratory chain targets with products at Assay Genie.

Gene and protein annotations from HGNC, NCBI Gene and UniProt P19404; product specifications from Assay Genie datasheets.

NDUFV2 Product listing