NDUFV1 抗体 (14.8): sc-100566

NDUFV1 Antibody (14.8) is an IgG_2b κ mouse monoclonal NDUFV1 antibody (also designated NDUFV1 antibody) that detects the NDUFV1 protein of human origin by WB, IP and ELISA. NDUFV1 Antibody (14.8) is available as the non-conjugated anti-NDUFV1 antibody. Complex 1 (also known as NADH dehydrogenase) of the electron transport chain (respiratory chain) is an enzymatic complex that catalyzes the transfer of electrons from NADH to ubiquinone. Free energy from the reaction is conserved in the transfer of protons into the inter-membrane space to create an electrochemical proton gradient, a driving force for ATP synthesis. Complex 1 is an extremely complicated, multi-protein, L-shaped complex composed of 45 different subunits located in the mitochondrial inner-membrane. NDUFV1 (NADH dehydrogenase ubiquinone flavoprotein 1), also known as UQOR1 or CI-51kD, is a 464 amino acid core subunit protein of complex 1 that is essential for the electron transport chain. The peripheral membrane protein, NDUFV1, contains a highly conserved NADH-binding site and is localized to the matrix side of the inner membrane. Defects in the gene encoding NDUFV1 can cause complex 1 deficiency in humans, which can to lead to Leigh syndrome (LS), a severe neurological disorder characterized by lesions in the subcortical brain region.

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References:

1. Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy.  |  Schuelke, M., et al. 1999. Nat Genet. 21: 260-1. PMID: 10080174
2. Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency.  |  Bεnit, P., et al. 2001. Am J Hum Genet. 68: 1344-52. PMID: 11349233
3. Mitochondrial complex I mutations in Caenorhabditis elegans produce cytochrome c oxidase deficiency, oxidative stress and vitamin-responsive lactic acidosis.  |  Grad, LI. and Lemire, BD. 2004. Hum Mol Genet. 13: 303-14. PMID: 14662656
4. The human mitochondrial NADH: ubiquinone oxidoreductase 51-kDa subunit maps adjacent to the glutathione S-transferase P1-1 gene on chromosome 11q13.  |  Spencer, SR., et al. 1992. Genomics. 14: 1116-8. PMID: 1478657
5. Chromosomal localization of the human gene encoding the 51-kDa subunit of mitochondrial complex I (NDUFV1) to 11q13.  |  Ali, ST., et al. 1993. Genomics. 18: 435-9. PMID: 8288251
6. Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3′UTR with the 5′UTR of the gamma-interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation?  |  Schuelke, M., et al. 1998. Biochem Biophys Res Commun. 245: 599-606. PMID: 9571201
7. The structure of the human NDUFV1 gene encoding the 51-kDa subunit of mitochondrial complex I.  |  de Coo, RF., et al. 1999. Mamm Genome. 10: 49-53. PMID: 9892733