NDUFS3

NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)

Identifiers

External IDs

OMIM: 603846 MGI: 1915599 HomoloGene: 3346 GeneCards: NDUFS3 Gene

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

RefSeq (mRNA)

RefSeq (protein)

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]

1 Function
2 See Also
3 References
4 Further reading

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]

References

^ 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.
^ ^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.

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.

NDUFS3

Contents

Function

See Also

References

Further reading