Rabbit anti-Human NDUFV1 Polyclonal Antibody

Uniprot Id

P49821

Target Species

Human

Target Name

NDUFV1

Target Full Name

NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial

Target Function

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor.

Target Involvement

Leigh syndrome (LS); Mitochondrial complex I deficiency (MT-C1D)

Target Subcellular Location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Target Protein Families

Complex I 51 kDa subunit family

Target Synonyms

CI 51kD; CI-51kD; CI51KD; Complex I 51kD; Complex I-51kD; FLJ59059; mitochondrial; NADH dehydrogenase (ubiquinone) flavoprotein 1; NADH dehydrogenase [ubiquinone] flavoprotein 1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; NADH dehydrogenase flavoprotein 1; NADH ubiquinone oxidoreductase 51 kDa subunit; NADH ubiquinone oxidoreductase; NADH ubiquinone oxidoreductase core subunit V1; NADH-ubiquinone oxidoreductase 51 kDa subunit; NDUFV 1; ndufv1; NDUV1_HUMAN; UQOR1

Target Background

The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10, 000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.