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NDUFV1 Full-Length MS Protein Standard

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Product Description


NameNDUFV1 Full-Length MS Protein Standard
Cat#CPFL304954
Protein NameNADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
SymbolNDUFV1
SynonymsCI-51K; CI51KD; UQOR1
AccessionNM_007103
Cytogenetic11q13
Amino Acid Labeled[U- 13C6, 15N4]-L-Arg and [U- 13C6, 15N2]-L-Lys
DescriptionNDUFV1 Full-Length MS Protein Standard (NP_009034), Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine, was produced in human 293 cells (HEK293) with fully chemically defined cell culture medium to obtain incorporation efficiency at Creative-Proteomics. 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.

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