Specifications
Immunogen
NDUFV1 (NP_002466, 365 a.a. ~ 464 a.a) partial recombinant protein with GST tag. The sequence is KAIARLIEFYKHESCGQCTPCREGVDWMNKVMARFVRGDARPAEIDSLWEISKQIEGHTI CALGDGAAWPVQGLIRHFRPELEERMQRFAQQHQARQAAS
Target
Alternative Names
NDUFV1; NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa; UQOR1; CI-51K; CI51KD; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; complex I 51kDa subunit; complex I 51 kda subunit; complex I, mitochondrial respiratory chain; NADH-ubiqui
Product Background
Antigen Description
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 Parkinsons disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009]
Pathway
Alzheimers disease; Electron Transport Chain; Huntingtons disease; NADH dehydrogenase (ubiquinone) Fe-S protein/flavoprotein complex, mitochondria
Citations
Publication ()
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