MT-ND6

NADH dehydrogenase, subunit 6 (complex I)
Identifiers
Symbols	ND6; MTND6
External IDs	OMIM: 516006 MGI: 102495 HomoloGene: 5022 GeneCards: ND6 Gene
Gene Ontology Molecular function • NADH dehydrogenase (ubiquinone) activity • oxidoreductase activity Cellular component • mitochondrion • mitochondrial inner membrane • mitochondrial respiratory chain complex I • membrane • integral to membrane • respiratory chain Biological process • mitochondrial electron transport, NADH to ubiquinone • transport • respiratory electron transport chain • response to hydrogen peroxide Sources: Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	4541	17722
Ensembl	ENSG00000198695	ENSMUSG00000064368
UniProt	P03923	Q7JCY4
RefSeq (mRNA)	n/a	n/a
RefSeq (protein)	YP_003024037.1	NP_904339.1
Location (UCSC)	Chr MT: 0.01 – 0.01 Mb	Chr MT: 0.01 – 0.01 Mb
PubMed search	[1]	[2]

NADH-ubiquinone oxidoreductase chain 6 is an enzyme that in humans is encoded by the MT-ND6 gene.

Further reading

• Torroni A, Achilli A, Macaulay V, et al. (2006). "Harvesting the fruit of the human mtDNA tree.". Trends Genet. 22 (6): 339–45. doi:10.1016/j.tig.2006.04.001. PMID 16678300. 
• Johns DR, Neufeld MJ, Park RD (1992). "An ND-6 mitochondrial DNA mutation associated with Leber hereditary optic neuropathy.". Biochem. Biophys. Res. Commun. 187 (3): 1551–7. doi:10.1016/0006-291X(92)90479-5. PMID 1417830. 
• Marzuki S, Noer AS, Lertrit P, et al. (1992). "Normal variants of human mitochondrial DNA and translation products: the building of a reference data base.". Hum. Genet. 88 (2): 139–45. PMID 1757091. 
• Moraes CT, Andreetta F, Bonilla E, et al. (1991). "Replication-competent human mitochondrial DNA lacking the heavy-strand promoter region". Mol. Cell. Biol. 11 (3): 1631–7. PMC 369459. PMID 1996112. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=369459. 
• Ozawa T, Tanaka M, Sugiyama S, et al. (1991). "Patients with idiopathic cardiomyopathy belong to the same mitochondrial DNA gene family of Parkinson's disease and mitochondrial encephalomyopathy". Biochem. Biophys. Res. Commun. 177 (1): 518–25. doi:10.1016/0006-291X(91)92014-B. PMID 2043137. 
• Chomyn A, Cleeter MW, Ragan CI, et al. (1986). "URF6, last unidentified reading frame of human mtDNA, codes for an NADH dehydrogenase subunit". Science 234 (4776): 614–8. doi:10.1126/science.3764430. PMID 3764430. 
• Chomyn A, Mariottini P, Cleeter MW, et al. (1985). "Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase". Nature 314 (6012): 592–7. doi:10.1038/314592a0. PMID 3921850. 
• Anderson S, Bankier AT, Barrell BG, et al. (1981). "Sequence and organization of the human mitochondrial genome". Nature 290 (5806): 457–65. doi:10.1038/290457a0. PMID 7219534. 
• Montoya J, Ojala D, Attardi G (1981). "Distinctive features of the 5'-terminal sequences of the human mitochondrial mRNAs". Nature 290 (5806): 465–70. doi:10.1038/290465a0. PMID 7219535. 
• Horai S, Hayasaka K, Kondo R, et al. (1995). "Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs". Proc. Natl. Acad. Sci. U.S.A. 92 (2): 532–6. doi:10.1073/pnas.92.2.532. PMC 42775. PMID 7530363. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=42775. 
• Jun AS, Brown MD, Wallace DC (1994). "A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia". Proc. Natl. Acad. Sci. U.S.A. 91 (13): 6206–10. doi:10.1073/pnas.91.13.6206. PMC 44167. PMID 8016139. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=44167. 
• De Vries DD, Went LN, Bruyn GW, et al. (1996). "Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia". Am. J. Hum. Genet. 58 (4): 703–11. PMC 1914692. PMID 8644732. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1914692. 
• Leo-Kottler B, Christ-Adler M, Baumann B, et al. (1996). "Leber's hereditary optic neuropathy: clinical and molecular genetic results obtained in a family with a new point mutation at nucleotide position 14498 in the ND 6 gene". German journal of ophthalmology 5 (4): 233–40. PMID 8854108. 
• Sudoyo H, Sitepu M, Malik S, et al. (1999). "Leber's hereditary optic neuropathy in Indonesia: two families with the mtDNA 11778G>A and 14484T>C mutations". Hum. Mutat. Suppl 1: S271–4. PMID 9452107. 
• Besch D, Leo-Kottler B, Zrenner E, Wissinger B (1999). "Leber's hereditary optic neuropathy: clinical and molecular genetic findings in a patient with a new mutation in the ND6 gene". Graefes Arch. Clin. Exp. Ophthalmol. 237 (9): 745–52. doi:10.1007/s004170050307. PMID 10447650. 
• Andrews RM, Kubacka I, Chinnery PF, et al. (1999). "Reanalysis and revision of the Cambridge reference sequence for human mitochondrial DNA". Nat. Genet. 23 (2): 147. doi:10.1038/13779. PMID 10508508. 
• Ingman M, Kaessmann H, Pääbo S, Gyllensten U (2001). "Mitochondrial genome variation and the origin of modern humans". Nature 408 (6813): 708–13. doi:10.1038/35047064. PMID 11130070. 
• Chinnery PF, Brown DT, Andrews RM, et al. (2001). "The mitochondrial ND6 gene is a hot spot for mutations that cause Leber's hereditary optic neuropathy". Brain 124 (Pt 1): 209–18. doi:10.1093/brain/124.1.209. PMID 11133798. 
• Finnilä S, Lehtonen MS, Majamaa K (2001). "Phylogenetic Network for European mtDNA". Am. J. Hum. Genet. 68 (6): 1475–84. doi:10.1086/320591. PMC 1226134. PMID 11349229. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1226134. 
• Maca-Meyer N, González AM, Larruga JM, et al. (2003). "Major genomic mitochondrial lineages delineate early human expansions". BMC Genet. 2: 13. doi:10.1186/1471-2156-2-13. PMC 55343. PMID 11553319. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=55343. 

External links

• GeneReviews/NCBI/NIH/UW entry on Mitochondrial DNA-Associated Leigh Syndrome and NARP