NDUFV1

NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
Identifiers
Symbols	NDUFV1; CI-51K; CI51KD; FLJ59059; UQOR1
External IDs	OMIM: 161015 MGI: 107851 HomoloGene: 5151 GeneCards: NDUFV1 Gene
EC number	1.6.5.3
Gene Ontology Molecular function • NADH dehydrogenase activity • NADH dehydrogenase (ubiquinone) activity • FMN binding • metal ion binding • NAD binding • 4 iron, 4 sulfur cluster binding Cellular component • mitochondrion • mitochondrial inner membrane • mitochondrial inner membrane • mitochondrial respiratory chain complex I • mitochondrial respiratory chain complex I • mitochondrial respiratory chain complex I • membrane • respiratory chain Biological process • mitochondrial electron transport, NADH to ubiquinone • mitochondrial electron transport, NADH to ubiquinone • transport • respiratory electron transport chain Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	4723	17995
Ensembl	ENSG00000167792	ENSMUSG00000037916
UniProt	P49821	Q91YT0
RefSeq (mRNA)	NM_001166102.1	NM_133666.2
RefSeq (protein)	NP_001159574.1	NP_598427.1
Location (UCSC)	Chr 11: 67.37 – 67.38 Mb	Chr 19: 4.01 – 4.01 Mb
PubMed search	[1]	[2]

NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial is an enzyme that in humans is encoded by the NDUFV1 gene.^[1][2]

The NDUFV1 gene encodes the 51-kD subunit of complex I (NADH:ubiquinone oxidoreductase) of the mitochondrial respiratory chain.^[2]

References

1. ^ Spencer SR, Taylor JB, Cowell IG, Xia CL, Pemble SE, Ketterer B (Feb 1993). "The human mitochondrial NADH: ubiquinone oxidoreductase 51-kDa subunit maps adjacent to the glutathione S-transferase P1-1 gene on chromosome 11q13". Genomics 14 (4): 1116–8. doi:10.1016/S0888-7543(05)80144-2. PMID 1478657. 
2. ^ ^{a b} "Entrez Gene: NDUFV1 NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4723. 

Further reading

• Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides.". Gene 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298. 
• Ali ST, Duncan AM, Schappert K et al. (1994). "Chromosomal localization of the human gene encoding the 51-kDa subunit of mitochondrial complex I (NDUFV1) to 11q13". Genomics 18 (2): 435–9. doi:10.1006/geno.1993.1493. PMID 8288251. 
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene 200 (1–2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149. 
• Schuelke M, Loeffen J, Mariman E et al. (1998). "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?". Biochem. Biophys. Res. Commun. 245 (2): 599–606. doi:10.1006/bbrc.1998.8486. PMID 9571201. 
• Loeffen JL, Triepels RH, van den Heuvel LP et al. (1999). "cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed". Biochem. Biophys. Res. Commun. 253 (2): 415–22. doi:10.1006/bbrc.1998.9786. PMID 9878551. 
• de Coo RF, Buddiger PA, Smeets HJ, van Oost BA (1999). "The structure of the human NDUFV1 gene encoding the 51-kDa subunit of mitochondrial complex I". Mamm. Genome 10 (1): 49–53. doi:10.1007/s003359900941. PMID 9892733. 
• Schuelke M, Smeitink J, Mariman E et al. (1999). "Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy". Nat. Genet. 21 (3): 260–1. doi:10.1038/6772. PMID 10080174. 
• Hu RM, Han ZG, Song HD et al. (2000). "Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning". Proc. Natl. Acad. Sci. U.S.A. 97 (17): 9543–8. doi:10.1073/pnas.160270997. PMC 16901. PMID 10931946. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=16901. 
• Triepels RH, Hanson BJ, van den Heuvel LP et al. (2001). "Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns". J. Biol. Chem. 276 (12): 8892–7. doi:10.1074/jbc.M009903200. PMID 11112787. 
• Brenner M, Johnson AB, Boespflug-Tanguy O et al. (2001). "Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with Alexander disease". Nat. Genet. 27 (1): 117–20. doi:10.1038/83679. PMID 11138011. 
• Bénit P, Chretien D, Kadhom N et al. (2001). "Large-Scale Deletion and Point Mutations of the Nuclear NDUFV1 and NDUFS1 Genes in Mitochondrial Complex I Deficiency". Am. J. Hum. Genet. 68 (6): 1344–52. doi:10.1086/320603. PMC 1226121. PMID 11349233. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1226121. 
• Strausberg RL, Feingold EA, Grouse LH et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=139241. 
• Ota T, Suzuki Y, Nishikawa T et al. (2004). "Complete sequencing and characterization of 21,243 full-length human cDNAs". Nat. Genet. 36 (1): 40–5. doi:10.1038/ng1285. PMID 14702039. 
• Gerhard DS, Wagner L, Feingold EA et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=528928. 
• Rual JF, Venkatesan K, Hao T et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.