NDUFS3

NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)
Identifiers
Symbols	NDUFS3;
External IDs	OMIM: 603846 MGI: 1915599 HomoloGene: 3346 GeneCards: NDUFS3 Gene
EC number	3.1.3.16
Gene Ontology Molecular function • NADH dehydrogenase activity • protein binding • NADH dehydrogenase (ubiquinone) activity • electron carrier activity Cellular component • mitochondrion • mitochondrial inner membrane • mitochondrial respiratory chain complex I • mitochondrial respiratory chain complex I • membrane • mitochondrial membrane • respiratory chain Biological process • mitochondrial electron transport, NADH to ubiquinone • transport • induction of apoptosis • respiratory electron transport chain • negative regulation of cell growth • reactive oxygen species metabolic process Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	4722	68349
Ensembl	ENSG00000213619	ENSMUSG00000005510
UniProt	O75489	Q8BTZ3
RefSeq (mRNA)	NM_004551	NM_026688.2
RefSeq (protein)	NP_004542	NP_080964.1
Location (UCSC)	Chr 11: 47.59 – 47.61 Mb	Chr 2: 90.73 – 90.74 Mb
PubMed search	[1]	[2]

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial is an enzyme that in humans is encoded by the NDUFS3 gene.^[1][2]

Function

The multisubunit NADH:ubiquinone oxidoreductase (complex I) is the first enzyme complex in the electron transport chain of mitochondria. The iron-sulfur protein (IP) fraction of complex I is made up of 7 subunits.^[2]

See Also

• NDUFS1

References

1. ^ Emahazion T, Beskow A, Gyllensten U, Brookes AJ (Nov 1998). "Intron based radiation hybrid mapping of 15 complex I genes of the human electron transport chain". Cytogenet Cell Genet 82 (1–2): 115–9. doi:10.1159/000015082. PMID 9763677. 
2. ^ ^{a b} "Entrez Gene: NDUFS3 NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4722. 

Further reading

• Dawson SJ, White LA (1992). "Treatment of Haemophilus aphrophilus endocarditis with ciprofloxacin". J. Infect. 24 (3): 317–20. doi:10.1016/S0163-4453(05)80037-4. PMID 1602151. 
• 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. 
• 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. 
• Loeffen J, van den Heuvel L, Smeets R, et al. (1998). "cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completed". Biochem. Biophys. Res. Commun. 247 (3): 751–8. doi:10.1006/bbrc.1998.8882. PMID 9647766. 
• 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. 
• 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. 
• Procaccio V, Lescuyer P, Bourges I, et al. (2000). "Human NDUFS3 gene coding for the 30-kDa subunit of mitochondrial complex I: genomic organization and expression". Mamm. Genome 11 (9): 808–10. doi:10.1007/s003350010160. PMID 10967146. 
• 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. 
• Kim SH, Fountoulakis M, Dierssen M, Lubec G (2002). "Decreased protein levels of complex I 30-kDa subunit in fetal Down syndrome brains". J. Neural Transm. Suppl. (61): 109–16. PMID 11771736. 
• 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. 
• Bénit P, Slama A, Cartault F, et al. (2004). "Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome". J. Med. Genet. 41 (1): 14–7. doi:10.1136/jmg.2003.014316. PMC 1757256. PMID 14729820. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1757256. 
• Bourges I, Ramus C, Mousson de Camaret B, et al. (2005). "Structural organization of mitochondrial human complex I: role of the ND4 and ND5 mitochondria-encoded subunits and interaction with prohibitin". Biochem. J. 383 (Pt. 3): 491–9. doi:10.1042/BJ20040256. PMC 1133742. PMID 15250827. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1133742. 
• 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. 
• Huang G, Chen Y, Lu H, Cao X (2007). "Coupling mitochondrial respiratory chain to cell death: an essential role of mitochondrial complex I in the interferon-beta and retinoic acid-induced cancer cell death". Cell Death Differ. 14 (2): 327–37. doi:10.1038/sj.cdd.4402004. PMID 16826196. 
• Vogel RO, Dieteren CE, van den Heuvel LP, et al. (2007). "Identification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunits". J. Biol. Chem. 282 (10): 7582–90. doi:10.1074/jbc.M609410200. PMID 17209039.