MT-CO3

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MT-CO3

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"COX3" redirects here. For the cyclooxygenase isoenzyme, see COX-3.

Cytochrome c oxidase III

Identifiers

Symbols

COX3; COIII; MTCO3

External IDs

OMIM: 516050 MGI: 102502 HomoloGene: 5014 GeneCards: COX3 Gene

EC number

1.9.3.1

Gene Ontology
Molecular function	• cytochrome-c oxidase activity
Cellular component	• mitochondrion • mitochondrial inner membrane • membrane • integral to membrane
Biological process	• mitochondrial electron transport, cytochrome c to oxygen • respiratory electron transport chain
Sources: Amigo / QuickGO

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

n/a

RefSeq (protein)

NP_536849

NP_904334.1

Location (UCSC)

Chr MT:
0.01 – 0.01 Mb

PubMed search

[1]

[2]

Cytochrome c oxidase subunit 3 is an enzyme that in humans is encoded by the MT-CO3 gene.^[1]

Contents

1 Clinical significance
2 References
3 Further reading
4 External links

Clinical significance

Mutations in mtDNA-encoded cytochrome c oxidase subunit genes have been observed to be associated with isolated myopathy or severe encephalomyopathy. ^[2]

References

^ "Entrez Gene: COX3 cytochrome c oxidase subunit III". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4514.
^ Horváth, R; Schoser, BG; Müller-Höcker, J; Völpel, M; Jaksch, M; Lochmüller, H (2005). "Mutations in mtDNA-encoded cytochrome c oxidase subunit genes causing isolated myopathy or severe encephalomyopathy". Neuromuscular disorders : NMD 15 (12): 851–7. doi:10.1016/j.nmd.2005.09.005. PMID 16288875.

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.
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.
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.
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.
Herrnstadt C, Elson JL, Fahy E, et al. (2002). "Reduced-Median-Network Analysis of Complete Mitochondrial DNA Coding-Region Sequences for the Major African, Asian, and European Haplogroups". Am. J. Hum. Genet. 70 (5): 1152–71. doi:10.1086/339933. PMC 447592. PMID 11938495. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=447592.
Silva WA, Bonatto SL, Holanda AJ, et al. (2002). "Mitochondrial Genome Diversity of Native Americans Supports a Single Early Entry of Founder Populations into America". Am. J. Hum. Genet. 71 (1): 187–92. doi:10.1086/341358. PMC 384978. PMID 12022039. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=384978.
Elkon H, Don J, Melamed E, et al. (2003). "Mutant and wild-type alpha-synuclein interact with mitochondrial cytochrome C oxidase". J. Mol. Neurosci. 18 (3): 229–38. doi:10.1385/JMN:18:3:229. PMID 12059041.
Mishmar D, Ruiz-Pesini E, Golik P, et al. (2003). "Natural selection shaped regional mtDNA variation in humans". Proc. Natl. Acad. Sci. U.S.A. 100 (1): 171–6. doi:10.1073/pnas.0136972100. PMC 140917. PMID 12509511. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=140917.
Ingman M, Gyllensten U (2003). "Mitochondrial Genome Variation and Evolutionary History of Australian and New Guinean Aborigines". Genome Res. 13 (7): 1600–6. doi:10.1101/gr.686603. PMC 403733. PMID 12840039. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=403733.
Kong QP, Yao YG, Sun C, et al. (2003). "Phylogeny of East Asian Mitochondrial DNA Lineages Inferred from Complete Sequences". Am. J. Hum. Genet. 73 (3): 671–6. doi:10.1086/377718. PMC 1180693. PMID 12870132. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1180693.
Temperley RJ, Seneca SH, Tonska K, et al. (2004). "Investigation of a pathogenic mtDNA microdeletion reveals a translation-dependent deadenylation decay pathway in human mitochondria". Hum. Mol. Genet. 12 (18): 2341–8. doi:10.1093/hmg/ddg238. PMID 12915481.
Maca-Meyer N, González AM, Pestano J, et al. (2004). "Mitochondrial DNA transit between West Asia and North Africa inferred from U6 phylogeography". BMC Genet. 4: 15. doi:10.1186/1471-2156-4-15. PMC 270091. PMID 14563219. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=270091.
Coble MD, Just RS, O'Callaghan JE, et al. (2004). "Single nucleotide polymorphisms over the entire mtDNA genome that increase the power of forensic testing in Caucasians". Int. J. Legal Med. 118 (3): 137–46. doi:10.1007/s00414-004-0427-6. PMID 14760490.
Palanichamy MG, Sun C, Agrawal S, et al. (2005). "Phylogeny of Mitochondrial DNA Macrohaplogroup N in India, Based on Complete Sequencing: Implications for the Peopling of South Asia". Am. J. Hum. Genet. 75 (6): 966–78. doi:10.1086/425871. PMC 1182158. PMID 15467980. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1182158.
Starikovskaya EB, Sukernik RI, Derbeneva OA, et al. (2005). "Mitochondrial DNA diversity in indigenous populations of the southern extent of Siberia, and the origins of Native American haplogroups". Ann. Hum. Genet. 69 (Pt 1): 67–89. doi:10.1046/j.1529-8817.2003.00127.x. PMID 15638829.
Rajkumar R, Banerjee J, Gunturi HB, et al. (2006). "Phylogeny and antiquity of M macrohaplogroup inferred from complete mt DNA sequence of Indian specific lineages". BMC Evol. Biol. 5: 26. doi:10.1186/1471-2148-5-26. PMC 1079809. PMID 15804362. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1079809.

External links

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

This protein-related article is a stub. You can help Wikipedia by expanding it.

v d e Mitochondrial proteins

Outer membrane	fatty acid degradation (Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase) tryptophan metabolism (Kynureninase) monoamine neurotransmitter metabolism (Monoamine oxidase)

Intermembrane space	Adenylate kinase - Creatine kinase

Inner membrane	oxidative phosphorylation (Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase) pyrimidine metabolism (Dihydroorotate dehydrogenase) mitochondrial shuttle (Malate-aspartate shuttle, Glycerol phosphate shuttle) other (Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II, Uncoupling protein)

Matrix	citric acid cycle (Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase) anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex) urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase) alcohol metabolism (ALDH2) PMPCB

Other/to be sorted	steroidogenesis (Cholesterol side-chain cleavage enzyme, Steroid 11-beta-hydroxylase, Aldosterone synthase), Frataxin Mitochondrial membrane transport protein (Mitochondrial permeability transition pore, Mitochondrial carrier)

Mitochondrial DNA	Complex I (MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6) - Complex III (MT-CYB) - Complex IV (MT-CO1, MT-CO2, MT-CO3) ATP synthase (MT-ATP6, MT-ATP8) tRNA (MT-TA, MT-TC, MT-TD, MT-TE, MT-TF, MT-TG, MT-TH, MT-TI, MT-TK, MT-TL1, MT-TL2, MT-TM, MT-TN, MT-TP, MT-TQ, MT-TR, MT-TS1, MT-TS2, MT-TT, MT-TV, MT-TW, MT-TY)

see also mitochondrial diseases B strc: edmb (perx), skel (ctrs), epit, cili, mito, nucl (chro)

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Wikipedia on Answers.com This article is licensed under the Creative Commons Attribution/Share-Alike License. It uses material from the Wikipedia article MT-CO3. Read