1777:. Panels A, B were cut across the long axes of the crypts and panels C, D were cut parallel to the long axes of the crypts. In panel A the bar shows 100 μm and allows an estimate of the frequency of crypts in the colonic epithelium. Panel B includes three crypts in cross-section, each with one segment deficient for MT-COI expression and at least one crypt, on the right side, undergoing fission into two crypts. Panel C shows, on the left side, a crypt fissioning into two crypts. Panel D shows typical small clusters of two and three MT-COI deficient crypts (the bar shows 50 μm). The images were made from original photomicrographs, but panels A, B and D were also included in an article and illustrations were published with Creative Commons Attribution-Noncommercial License allowing re-use.
1754:
214:
1164:
1347:
well as a low-spin heme, both ligated to six conserved histidine residues near the outer side of four transmembrane spans within COI is common to all family members. In contrast to eukaryotes the respiratory chain of prokaryotes is branched to multiple terminal oxidases. The enzyme complexes vary in heme and copper composition, substrate type and substrate affinity. The different respiratory oxidases allow the cells to customize their respiratory systems according to a variety of environmental growth conditions.
1843:). There are about 100 to 700 mitochondria per cell, depending on cell type. Furthermore, there is fairly rapid turnover of mitochondria, so that a mitochondrion with MT-COI-mutated chromosomes and a positive selection bias could shortly become the major type of mitochondrion in a cell. The average half-life of mitochondria in rats, depending on cell type, is between 9 and 24 days, and in mice is about 2 days. In humans it is likely that the half life of mitochondria is also a matter of days to weeks.
1855:
the same cell to generate homoplasmy for MT-COI-deficiency. Another suggestion was that cells with a deficiency in cytochrome c oxidase are apoptosis resistant, and thus more likely to survive. The linkage of MT-COI to apoptosis arises because active cytochrome c oxidase oxidizes cytochrome c, which then activates pro-caspase 9, leading to apoptosis. These two factors may contribute to the frequent occurrence of MT-COI-deficient colonic crypts with age or during carcinogenesis in the human colon.
134:
4664:
4649:
4694:
4679:
2153:
4514:
4529:
4709:
4604:
4574:
4634:
4589:
4544:
4559:
4619:
972:
1809:
Greaves et al. showed that deficiencies of MT-COI in colonic crypts are due to mutations in the MT-COI gene. As seen in panel B, a portion of the stem cells of three crypts appear to have a mutation in MT-COI, so that 40% to 50% of the cells arising from those stem cells form a white segment in the cross-cut area.
1854:
It is not clear why a deficiency of MT-COI should have a positive selective bias. One suggestion is that deficiency of MT-COI in a mitochondrion leads to lower reactive oxygen production (and less oxidative damage) and this provides a selective advantage in competition with other mitochondria within
1846:
A stem cell at the base of a colonic crypt that was largely MT-COI-deficient may compete with the other 4 or 5 stem cells to take over the stem cell niche. If this occurs, then the colonic crypt would be deficient in MT-COI in all 1700 to 5,000 cells, as is indicated for some crypts in panels A, B
1850:
Crypts of the colon can reproduce by fission, as seen in panel C, where a crypt is fissioning to form two crypts, and in panel B where at least one crypt appears to be fissioning. Most crypts deficient in MT-COI are in clusters of crypts (clones of crypts) with two or more MT-COI-deficient crypts
1820:
The occurrence of frequent crypts with almost complete loss of MT-COI in their 1700 to 5,000 cells suggests a process of natural selection. However, it has also been shown that a deficiency throughout a particular crypt due to an initial mitochondrial DNA mutation may occasionally occur through a
1346:
The enzyme complex consists of 3-4 subunits (prokaryotes) up to 13 polypeptides (mammals) of which only the catalytic subunit (equivalent to mammalian subunit I (COI)) is found in all heme-copper respiratory oxidases. The presence of a bimetallic centre (formed by a high-spin heme and copper B) as
1808:
Most of the human colonic crypts in the images have high expression of the brown-orange stained MT-COI. However, in some of the colonic crypts all of the cells lack MT-COI and appear mostly white, with their main color being the blue-gray staining of the nuclei at the outer walls of the crypts.
1812:
In humans, the percent of colonic crypts deficient for MT-COI is less than 1% before age 40, but then increases linearly with age. On average, the percent of colonic crypts deficient for MT-COI reaches 18% in women and 23% in men by 80–84 years of age. Colonic tumors often arise in a field of
1816:
As seen in panels C and D, crypts are about 75 to about 110 cells long. The average crypt circumference is 23 cells. Based on these measurements, crypts have between 1725 and 2530 cells. Another report gave a range of 1500 to 4900 cells per colonic crypt.
1724:
sequence differences are too small to be detected between closely related species, more than 2% sequence divergence is typically detected between closely related animal species, suggesting that the barcode is effective for most animals. In most if not all
1343:), is directly involved in the coupling between dioxygen reduction and proton pumping. Some terminal oxidases generate a transmembrane proton gradient across the plasma membrane (prokaryotes) or the mitochondrial inner membrane (eukaryotes).
1828:. There are multiple copies of the chromosome in most mitochondria, usually between 2 and 6 per mitochondrion. If a mutation occurs in MT-COI in one chromosome of a mitochondrion, there may be random segregation of the chromosomes during
3957:
Lu X, Walker T, MacManus JP, Seligy VL (July 1992). "Differentiation of HT-29 human colonic adenocarcinoma cells correlates with increased expression of mitochondrial RNA: effects of trehalose on cell growth and maturation".
1851:
adjacent to each other (see panel D). This illustrates that clones of deficient crypts often arise, and thus that there is likely a positive selective bias that has allowed them to spread in the human colonic epithelium.
4122:
Chomyn A, Mariottini P, Cleeter MW, Ragan CI, Matsuno-Yagi A, Hatefi Y, et al. (1985). "Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase".
2785:
Lucioli S, Hoffmeier K, Carrozzo R, Tessa A, Ludwig B, Santorelli FM (March 2006). "Introducing a novel human mtDNA mutation into the
Paracoccus denitrificans COX I gene explains functional deficits in a patient".
1800:
inner surface of the colon is punctuated by invaginations, the colonic crypts. The colon crypts are shaped like microscopic thick walled test tubes with a central hole down the length of the tube (the crypt
3979:
Marzuki S, Noer AS, Lertrit P, Thyagarajan D, Kapsa R, Utthanaphol P, Byrne E (December 1991). "Normal variants of human mitochondrial DNA and translation products: the building of a reference data base".
1793:(colon). However, MT-COI is frequently lost in colonic crypts with age in humans and is also often absent in field defects that give rise to colon cancers as well as in portions of colon cancers.
1496:
Mutations in this gene can cause mitochondrial
Complex IV deficiency, a disease of the mitochondrial respiratory chain displaying a wide variety of clinical manifestations ranging from isolated
1821:
stochastic process. Nevertheless, the frequent occurrence of MT-COI deficiency in many crypts within a colon epithelium indicates that absence of MT-COI likely provides a selective advantage.
1606:(necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, exercise intolerance, low muscle capacity for oxidative phosphorylation, and followed by excretion of
4060:
Attardi G, Chomyn A, Doolittle RF, Mariottini P, Ragan CI (1987). "Seven unidentified reading frames of human mitochondrial DNA encode subunits of the respiratory chain NADH dehydrogenase".
2947:
Karadimas CL, Greenstein P, Sue CM, Joseph JT, Tanji K, Haller RG, et al. (September 2000). "Recurrent myoglobinuria due to a nonsense mutation in the COX I gene of mitochondrial DNA".
1376:) exists in denitrifying species of archaea and eubacteria and is a heterodimer of cytochromes b and c. Phenazine methosulphate can act as acceptor. It has been suggested that cytochrome
4663:
4648:
1990:
Tsukihara T, Aoyama H, Yamashita E, Tomizaki T, Yamaguchi H, Shinzawa-Itoh K, et al. (May 1996). "The whole structure of the 13-subunit oxidized cytochrome c oxidase at 2.8 A".
4693:
2709:
Bröker S, Meunier B, Rich P, Gattermann N, Hofhaus G (November 1998). "MtDNA mutations associated with sideroblastic anaemia cause a defect of mitochondrial cytochrome c oxidase".
4678:
4085:
Chomyn A, Cleeter MW, Ragan CI, Riley M, Doolittle RF, Attardi G (October 1986). "URF6, last unidentified reading frame of human mtDNA, codes for an NADH dehydrogenase subunit".
1630:. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies. The mutation, A1555G, has been associated with this disease.
4749:
1131:
2059:
4513:
1465:
may be involved in the development of acquired idiopathic sideroblastic anemia. Mutations in mitochondrial DNA can cause respiratory chain dysfunction, preventing
1075:
1063:
3924:"A mitochondrial DNA variant, identified in Leber hereditary optic neuropathy patients, which extends the amino acid sequence of cytochrome c oxidase subunit I"
2629:"A mitochondrial DNA variant, identified in Leber hereditary optic neuropathy patients, which extends the amino acid sequence of cytochrome c oxidase subunit I"
2670:"Heteroplasmic point mutations of mitochondrial DNA affecting subunit I of cytochrome c oxidase in two patients with acquired idiopathic sideroblastic anemia"
5549:
5230:
4528:
1813:
crypts containing a large cluster (as many as 410) of MT-COI-deficient crypts. In colonic cancers, up to 80% of tumor cells can be deficient in MT-COI.
709:
690:
95:
4708:
4603:
4573:
4633:
4588:
4543:
1354:
oxidase in Gram-positive bacteria and that archaebacterial quinol oxidase has an independent origin. A considerable amount of evidence suggests that
5225:
5316:
4898:
3391:
Coller HA, Bodyak ND, Khrapko K (April 2002). "Frequent intracellular clonal expansions of somatic mtDNA mutations: significance and mechanisms".
1805:). Four tissue sections are shown in the image in this section, two cut across the long axes of the crypts and two cut parallel to the long axes.
4168:
Sanger F, Coulson AR, Barrell BG, Smith AJ, Roe BA (October 1980). "Cloning in single-stranded bacteriophage as an aid to rapid DNA sequencing".
2060:"COI barcoding of Nebelid testate amoebae (Amoebozoa: Arcellinida): extensive cryptic diversity and redefinition of the Hyalospheniidae Schultze"
4984:
4558:
1392:
1232:
3344:"Age-associated mitochondrial DNA mutations lead to small but significant changes in cell proliferation and apoptosis in human colonic crypts"
3033:"Heterogenous point mutations in the mitochondrial tRNA Ser(UCN) precursor coexisting with the A1555G mutation in deaf students from Mongolia"
4493:
1972:
1031:
4793:
1954:
4742:
4618:
1333:
2992:"Mitochondrial myopathy and rhabdomyolysis associated with a novel nonsense mutation in the gene encoding cytochrome c oxidase subunit I"
213:
2102:
66:, mitochondrially encoded cytochrome c oxidase I, COI, MTCO1, Main subunit of cytochrome c oxidase, CO I, cytochrome c oxidase subunit I
5569:
5084:
5019:
5029:
4854:
1681:
4197:
Montoya J, Ojala D, Attardi G (April 1981). "Distinctive features of the 5'-terminal sequences of the human mitochondrial mRNAs".
3671:"Mitochondrial turnover in liver is fast in vivo and is accelerated by dietary restriction: application of a simple dynamic model"
5355:
5153:
4788:
3547:
Robin ED, Wong R (September 1988). "Mitochondrial DNA molecules and virtual number of mitochondria per cell in mammalian cells".
133:
4338:
Ingman M, Kaessmann H, Pääbo S, Gyllensten U (December 2000). "Mitochondrial genome variation and the origin of modern humans".
5584:
5522:
5350:
5014:
4735:
1941:
1920:
1653:
1639:
1667:
5131:
5089:
3893:
Bodenteich A, Mitchell LG, Polymeropoulos MH, Merril CR (May 1992). "Dinucleotide repeat in the human mitochondrial D-loop".
3593:
Satoh M, Kuroiwa T (September 1991). "Organization of multiple nucleoids and DNA molecules in mitochondria of a human cell".
903:
1937:
1151:
910:
1832:
to generate new mitochondria. This can give rise to a mitochondrion with primarily or solely MT-COI-mutated chromosomes.
1916:
1720:
and also because its sequence is conserved among conspecifics. Contrary to the primary objection raised by skeptics that
4935:
4877:
4767:
1868:
75:
4989:
3864:
Torroni A, Achilli A, Macaulay V, Richards M, Bandelt HJ (June 2006). "Harvesting the fruit of the human mtDNA tree".
1742:
1533:
3236:"Mitochondrial DNA mutations are established in human colonic stem cells, and mutated clones expand by crypt fission"
2831:"Mitochondrial DNA mutations are established in human colonic stem cells, and mutated clones expand by crypt fission"
3501:
Legros F, Malka F, Frachon P, Lombès A, Rojo M (June 2004). "Organization and dynamics of human mitochondrial DNA".
1380:
oxidase catalytic subunits evolved from ancient nitric oxide reductases that could reduce both nitrogen and oxygen.
5163:
5158:
4962:
4828:
1529:
1753:
5579:
4888:
4486:
1773:
of the cells (located at the outer edges of the cells lining the walls of the crypts) are stained blue-gray with
1318:
1087:
147:
1610:
in the urine. It has been associated with mitochondrial myopathy. A G5920A mutation, and a heteroplasmic G6708A
5574:
5079:
4957:
1447:
1370:
1358:(also known as proteobacteria or purple bacteria) acquired quinol oxidase through a lateral gene transfer from
1337:
1314:
1307:
1303:
754:
83:
1139:
1324:
Proton pumping heme-copper oxidases represent the terminal, energy-transfer enzymes of respiratory chains in
5126:
4913:
1525:
1451:
735:
1163:
5121:
4778:
1575:
1411:
1366:
1359:
1332:. The CuB-heme a3 (or heme o) binuclear centre, associated with the largest subunit I of cytochrome c and
1251:
3761:"MITRAC7 Acts as a COX1-Specific Chaperone and Reveals a Checkpoint during Cytochrome c Oxidase Assembly"
5495:
5235:
5111:
4947:
4925:
3442:
Nekhaeva E, Bodyak ND, Kraytsberg Y, McGrath SB, Van Orsouw NJ, Pluzhnikov A, et al. (April 2002).
3342:
Nooteboom M, Johnson R, Taylor RW, Wright NA, Lightowlers RN, Kirkwood TB, et al. (February 2010).
1829:
1623:
1466:
1439:
1863:
Within the MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase)
1135:
4244:"Recent African origin of modern humans revealed by complete sequences of hominoid mitochondrial DNAs"
3293:
Baker AM, Cereser B, Melton S, Fletcher AG, Rodriguez-Justo M, Tadrous PJ, et al. (August 2014).
5336:
5242:
5099:
5048:
4994:
4479:
4347:
4255:
4206:
4132:
4094:
3455:
3400:
3247:
3183:
Bernstein C, Facista A, Nguyen H, Zaitlin B, Hassounah N, Loustaunau C, et al. (December 2010).
3031:
Pandya A, Xia XJ, Erdenetungalag R, Amendola M, Landa B, Radnaabazar J, et al. (December 1999).
2901:
2890:"A mitochondrial DNA mutation linked to colon cancer results in proton leaks in cytochrome c oxidase"
2842:
1999:
1839:
in order to frequently become the main type of mitochondrion in a cell (a cell with MT-COI-deficient
1762:
1627:
1517:
1403:
1399:
1299:
1243:
1239:
1228:
1044:
1092:
62:
2450:
1883:. This interaction with SMIM20 stabilizes the newly synthesized MT-CO1 and prevents its premature
5266:
5034:
4908:
4371:
4326:
4230:
4156:
4005:
3759:
Dennerlein S, Oeljeklaus S, Jans D, Hellwig C, Bareth B, Jakobs S, et al. (September 2015).
3572:
3526:
3424:
3082:"Barcoding animal life: cytochrome c oxidase subunit 1 divergences among closely related species"
2972:
2811:
2568:
2382:
2023:
1583:
1396:
1236:
107:
4672:: Cadmium ion binding structure of bovine heart cytochrome C oxidase in the fully oxidized state
3234:
Greaves LC, Preston SL, Tadrous PJ, Taylor RW, Barron MJ, Oukrif D, et al. (January 2006).
2829:
Greaves LC, Preston SL, Tadrous PJ, Taylor RW, Barron MJ, Oukrif D, et al. (January 2006).
2357:
Papa S, Capitanio N, Glaser P, Villani G (May 1994). "The proton pump of heme-copper oxidases".
886:
4657:: Cadmium ion binding structure of bovine heart cytochrome C oxidase in the fully reduced state
2744:
Varlamov DA, Kudin AP, Vielhaber S, Schröder R, Sassen R, Becker A, et al. (August 2002).
5256:
5059:
4840:
4459:
4414:
4363:
4318:
4283:
4222:
4185:
4148:
4110:
4073:
4048:
3997:
3967:
3945:
3910:
3881:
3841:
3810:
Lorenzi I, Oeljeklaus S, Aich A, Ronsör C, Callegari S, Dudek J, et al. (February 2018).
3792:
3741:
3700:
3651:
3610:
3564:
3518:
3483:
3416:
3373:
3324:
3275:
3216:
3152:
3111:
3062:
3013:
2964:
2929:
2870:
2803:
2767:
2726:
2691:
2668:
Gattermann N, Retzlaff S, Wang YL, Hofhaus G, Heinisch J, Aul C, Schneider W (December 1997).
2650:
2609:
2560:
2519:
2470:
2431:
2374:
2339:
2267:
2218:
2082:
2015:
1825:
1758:
1738:
1611:
1435:
1407:
1287:
1271:
1247:
1126:
55:
4702:: Zinc ion binding structure of bovine heart cytochrome C oxidase in the fully oxidized state
1888:
1880:
5069:
4862:
4687:: Zinc ion binding structure of bovine heart cytochrome C oxidase in the fully reduced state
4449:
4439:
4404:
4396:
4355:
4308:
4273:
4263:
4214:
4177:
4140:
4102:
4065:
4038:
4030:
3989:
3935:
3902:
3873:
3831:
3823:
3782:
3772:
3731:
3690:
3682:
3641:
3602:
3556:
3510:
3473:
3463:
3408:
3363:
3355:
3314:
3306:
3265:
3255:
3206:
3196:
3142:
3101:
3093:
3052:
3044:
3003:
2956:
2919:
2909:
2860:
2850:
2795:
2757:
2718:
2681:
2640:
2599:
2588:"Denitrification and aerobic respiration, hybrid electron transport chains and co-evolution"
2550:
2509:
2501:
2462:
2421:
2413:
2366:
2329:
2321:
2257:
2249:
2208:
2200:
2136:"MT-CO1 - Cytochrome c oxidase subunit 1 - Homo sapiens (Human) - MT-CO1 gene & protein"
2074:
2007:
1884:
1786:
1587:
1500:
to a severe multisystem disease affecting multiple organs and tissues. Symptoms may include
1431:
306:
237:
204:
159:
31:
3444:"Clonally expanded mtDNA point mutations are abundant in individual cells of human tissues"
1118:
4974:
4867:
4295:
Andrews RM, Kubacka I, Chinnery PF, Lightowlers RN, Turnbull DM, Howell N (October 1999).
3787:
3295:"Quantification of crypt and stem cell evolution in the normal and neoplastic human colon"
2157:
2152:
1864:
1802:
1790:
1563:
1513:
281:
103:
4297:"Reanalysis and revision of the Cambridge reference sequence for human mitochondrial DNA"
4351:
4259:
4210:
4136:
4098:
4019:"Replication-competent human mitochondrial DNA lacking the heavy-strand promoter region"
3459:
3404:
3251:
2905:
2846:
2003:
1488:. The result is a ferric accumulation in mitochondria and insufficient heme production.
4409:
4384:
3940:
3923:
3836:
3811:
3695:
3670:
3412:
3368:
3343:
3319:
3294:
3270:
3235:
3211:
3184:
3106:
3081:
3057:
3032:
2924:
2889:
2865:
2830:
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2628:
2505:
2417:
2262:
2237:
2213:
2188:
1836:
1603:
1579:
1541:
1446:
defects. Because this disease is a result of mitochondrial DNA mutations affecting the
1355:
4043:
4018:
3646:
3629:
3478:
3443:
2514:
2489:
2426:
2401:
2334:
2309:
1835:
A mitochondrion with largely MT-COI-mutated chromosomes would need to have a positive
624:
619:
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609:
604:
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584:
568:
563:
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4758:
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4243:
4181:
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3606:
3359:
3147:
3130:
2555:
2538:
2466:
2325:
2285:
2238:"Integration of cardiac proteome biology and medicine by a specialized knowledgebase"
1766:
1705:
1501:
1415:
1255:
1068:
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484:
4375:
4330:
4160:
4009:
3530:
3428:
2976:
2815:
2386:
2027:
1781:
The MT-COI protein, also known as CCOI, is usually expressed at a high level in the
1175:
is one of the three cytochrome c oxidase subunit mitochondrial genes (orange boxes).
1000:
5391:
5366:
5024:
4903:
4816:
4234:
3630:"The turnover of mitochondria in a variety of tissues of young adult and aged rats"
3576:
2572:
1774:
1770:
1505:
1114:
299:
124:
87:
2253:
2011:
1080:
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2078:
1689:
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1313:) is a key enzyme in aerobic metabolism. It is the third and final enzyme of the
1056:
1012:
111:
30:"Cox1" redirects here. Particularly in a medical context, this can also refer to
4069:
4017:
Moraes CT, Andreetta F, Bonilla E, Shanske S, DiMauro S, Schon EA (March 1991).
1977:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1959:
National Center for
Biotechnology Information, U.S. National Library of Medicine
1591:
1537:
1521:
1443:
1275:
1266:
In humans, the MT-CO1 gene is located from nucleotide pairs 5904 to 7444 on the
4248:
Proceedings of the
National Academy of Sciences of the United States of America
3448:
Proceedings of the
National Academy of Sciences of the United States of America
3240:
Proceedings of the
National Academy of Sciences of the United States of America
2894:
Proceedings of the
National Academy of Sciences of the United States of America
2835:
Proceedings of the
National Academy of Sciences of the United States of America
1570:. Numerous such genetic alterations are often involved with the progression of
382:
5143:
4805:
3877:
2799:
2686:
2669:
1840:
1797:
1726:
1556:
1555:
mutations play a role in colorectal cancer, a very complex disease displaying
1350:
It has been shown that eubacterial quinol oxidase was derived from cytochrome
1325:
221:
141:
91:
3185:"Cancer and age related colonic crypt deficiencies in cytochrome c oxidase I"
2762:
2745:
2236:
Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, et al. (October 2013).
2058:
Kosakyan A, Heger TJ, Leander BS, Todorov M, Mitchell EA, Lara E (May 2012).
5074:
4522:: STRUCTURE OF BOVINE HEART CYTOCHROME C OXIDASE AT THE FULLY OXIDIZED STATE
4106:
3922:
Brown MD, Yang CC, Trounce I, Torroni A, Lott MT, Wallace DC (August 1992).
3906:
3260:
3201:
3008:
2991:
2914:
2855:
2746:"Metabolic consequences of a novel missense mutation of the mtDNA CO I gene"
2627:
Brown MD, Yang CC, Trounce I, Torroni A, Lott MT, Wallace DC (August 1992).
2308:
García-Horsman JA, Barquera B, Rumbley J, Ma J, Gennis RB (September 1994).
1782:
1607:
1509:
1481:
1329:
1206:
654:
442:
320:
265:
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187:
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99:
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3468:
3420:
3377:
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3156:
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3017:
2968:
2933:
2874:
2807:
2771:
2613:
2370:
2271:
2222:
2086:
1769:
protein cytochrome c oxidase subunit I (CCOI, synonym for MT-COI), and the
4287:
4268:
4226:
4189:
4152:
4114:
4077:
4052:
4034:
4001:
3971:
3949:
3914:
3655:
3614:
3568:
2730:
2695:
2654:
2564:
2523:
2474:
2435:
2402:"Evolution of cytochrome oxidase, an enzyme older than atmospheric oxygen"
2378:
2343:
2204:
2019:
1040:
971:
950:
945:
5538:
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5094:
4727:
3131:"Potentiality of the cox1 gene in the taxonomic resolution of soil fungi"
3129:
Molitor C, Inthavong B, Sage L, Geremia RA, Mouhamadou B (January 2010).
2960:
2135:
2044:
1713:
1684:
1670:
1656:
1642:
1497:
1388:
1340:
1007:
934:
799:
780:
3812:"The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis"
2990:
Kollberg G, Moslemi AR, Lindberg C, Holme E, Oldfors A (February 2005).
1373:
5380:
5375:
5295:
4428:"Major genomic mitochondrial lineages delineate early human expansions"
3993:
1717:
1571:
1477:
1310:
1267:
1195:
1024:
1019:
766:
721:
3514:
2539:"Cytochrome oxidase evolved by tinkering with denitrification enzymes"
5485:
5480:
5450:
5445:
5325:
5305:
5300:
5290:
5285:
5280:
5275:
4359:
4218:
4144:
1876:
1567:
1559:
1470:
1295:
1291:
1146:
918:
676:
4426:
Maca-Meyer N, González AM, Larruga JM, Flores C, Cabrera VM (2003).
4242:
Horai S, Hayasaka K, Kondo R, Tsugane K, Takahata N (January 1995).
1536:. In some patients, the hypertrophic cardiomyopathy is fatal at the
17:
4400:
3048:
27:
Enzyme of the respiratory chain encoded by the mitochondrial genome
5505:
5500:
5490:
5475:
5470:
5465:
5460:
5455:
5440:
5435:
5430:
5425:
5420:
5415:
5410:
5405:
5400:
5196:
5183:
4537:: BOVINE HEART CYTOCHROME C OXIDASE IN CARBON MONOXIDE-BOUND STATE
4313:
4296:
1892:
1231:
complex. In humans, mutations in MT-CO1 have been associated with
1602:
RM-MT is a disease that is characterized by recurrent attacks of
639:
635:
5534:
2106:
1872:
1485:
1473:
1202:
1108:
1051:
995:
79:
4731:
4717:: BOVINE HEART CYTOCHROME C OXIDASE AT THE FULLY OXIDIZED STATE
4612:: Bovine heart cytochrome C oxidase at the fully oxidized state
4582:: Bovine heart cytochrome c oxidase at the fully oxidized state
4475:
1761:) within four tissue sections. The cells have been stained by
1286:
Cytochrome c oxidase subunit I (CO1 or MT-CO1) is one of three
4642:: Bovine heart cytochrome C oxidase in the fully reduced state
4597:: Bovine heart cytochrome c oxidase at the fully reduced state
4552:: BOVINE HEART CYTOCHROME C OXIDASE IN THE FULLY REDUCED STATE
4471:
2490:"Structural models of the redox centres in cytochrome oxidase"
2455:
Biochimica et
Biophysica Acta (BBA) - Reviews on Bioenergetics
3816:
Biochimica et Biophysica Acta (BBA) - Molecular Cell Research
1716:
rate is generally fast enough to distinguish closely related
3720:"Regulation of apoptosis by the redox state of cytochrome c"
1480:
iron, which is required for the final step in mitochondrial
2400:
Castresana J, Lübben M, Saraste M, Higgins DG (June 1994).
976:
Structure of the 13-subunit oxidized cytochrome c oxidase.
2156: This article incorporates text available under the
1745:(the gene most commonly used for mycological barcoding).
4567:: BOVINE HEART CYTOCHROME C OXIDASE IN AZIDE-BOUND STATE
615:
mitochondrial electron transport, cytochrome c to oxygen
289:
2290:
Cardiac Organellar Protein Atlas Knowledgebase (COPaKB)
1274:. The gene product is a 57 kDa protein composed of 513
5533:
This article incorporates text from the public domain
1765:
to show a brown-orange color if the cells produce the
3080:
Hebert PD, Ratnasingham S, deWaard JR (August 2003).
2310:"The superfamily of heme-copper respiratory oxidases"
454:
4627:: Bovine heart cytochrome C oxidase modified by DCCD
3669:
Miwa S, Lawless C, von Zglinicki T (December 2008).
2996:
Journal of Neuropathology and Experimental Neurology
1712:
gene sequence is suitable for this role because its
1614:
have been associated with COX deficiency and RM-MT.
1227:. Cytochrome c oxidase I is the main subunit of the
5389:
5364:
5334:
5314:
5264:
5255:
5209:
5172:
5140:
5108:
5056:
5047:
5003:
4971:
4944:
4922:
4885:
4876:
4853:
4825:
4802:
4775:
4766:
4062:
Cold Spring Harbor Symposia on Quantitative Biology
3724:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
2592:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
1145:
1125:
1107:
1102:
1086:
1074:
1062:
1050:
1030:
1018:
1006:
994:
986:
981:
964:
879:
860:
836:
817:
393:
dentate gyrus of hippocampal formation granule cell
2453:(July 1983). "Structure of cytochrome c oxidase".
1933:
1931:
1929:
1912:
1910:
1908:
1421:
236:
158:
2488:Holm L, Saraste M, Wikström M (September 1987).
4383:Finnilä S, Lehtonen MS, Majamaa K (June 2001).
1540:stage. Other affected individuals may manifest
2303:
2301:
2299:
2189:"UniProt: the universal protein knowledgebase"
1938:GRCm38: Ensembl release 89: ENSMUSG00000064351
1733:is very slow. It has also been suggested that
1682:Cytochrome c oxidase, subunit I bacterial type
1188:mitochondrially encoded cytochrome c oxidase I
4743:
4487:
2045:"Entrez Gene: Cytochrome c oxidase subunit I"
1618:Deafness, sensorineural, mitochondrial (DFNM)
1598:Recurrent myoglobinuria mitochondrial (RM-MT)
8:
3178:
3176:
3174:
3172:
3170:
3168:
3166:
1492:Mitochondrial Complex IV deficiency (MT-C4D)
1917:GRCh38: Ensembl release 89: ENSG00000198804
1391:in this gene in humans are associated with
625:electron transport coupled proton transport
564:mitochondrial respiratory chain complex III
5550:United States National Library of Medicine
5261:
5231:Mitochondrial permeability transition pore
5213:
5053:
4882:
4772:
4750:
4736:
4728:
4494:
4480:
4472:
3393:Annals of the New York Academy of Sciences
3189:World Journal of Gastrointestinal Oncology
1737:may be a better gene for DNA barcoding of
1422:Leber's hereditary optic neuropathy (LHON)
1099:
970:
650:
569:mitochondrial respiratory chain complex IV
480:
277:
199:
119:
4453:
4443:
4408:
4385:"Phylogenetic network for European mtDNA"
4312:
4277:
4267:
4042:
3939:
3835:
3786:
3776:
3735:
3694:
3645:
3477:
3467:
3367:
3318:
3269:
3259:
3210:
3200:
3146:
3105:
3056:
3007:
2923:
2913:
2864:
2854:
2761:
2685:
2644:
2603:
2554:
2513:
2425:
2333:
2286:"MT-CO1 - Cytochrome c oxidase subunit 1"
2261:
2212:
1654:Cytochrome o ubiquinol oxidase, subunit I
1640:Cytochrome c oxidase cbb3-type, subunit I
5548:This article incorporates text from the
5226:Mitochondrial membrane transport protein
3588:
3586:
3542:
3540:
2888:Namslauer I, Brzezinski P (March 2009).
2039:
2037:
1752:
1668:Cytochrome aa3 quinol oxidase, subunit I
1458:Acquired Idiopathic Sideroblastic Anemia
1171:gene in the human mitochondrial genome.
1162:
4509:
1904:
4985:Cholesterol side-chain cleavage enzyme
2537:Saraste M, Castresana J (March 1994).
961:
36:
2183:
2181:
2179:
2177:
2175:
2173:
2171:
2169:
2167:
241:
225:
220:
163:
145:
140:
7:
2130:
2128:
2126:
2124:
2122:
2120:
2118:
2116:
2114:
2098:
2096:
1729:, however, the rate of evolution of
1434:, causing subacute or acute central
4899:Coenzyme Q – cytochrome c reductase
3634:The Journal of Biological Chemistry
2103:Online Mendelian Inheritance in Man
1426:LHON, correlated with mutations in
1393:Leber's hereditary optic neuropathy
1233:Leber's hereditary optic neuropathy
5085:Oxoglutarate dehydrogenase complex
5020:Glycerol-3-phosphate dehydrogenase
4389:American Journal of Human Genetics
3928:American Journal of Human Genetics
3628:Menzies RA, Gold PH (April 1971).
3413:10.1111/j.1749-6632.2002.tb02113.x
3037:American Journal of Human Genetics
2723:10.1046/j.1432-1327.1998.2580132.x
2633:American Journal of Human Genetics
2586:Chen J, Strous M (February 2013).
2506:10.1002/j.1460-2075.1987.tb02578.x
2418:10.1002/j.1460-2075.1994.tb06541.x
1887:. Additionally, it interacts with
1704:is a gene that is often used as a
1290:(mtDNA) encoded subunits (MT-CO1,
876:
857:
833:
814:
790:
771:
745:
726:
700:
681:
459:
377:
315:
294:
25:
5030:Carnitine palmitoyltransferase II
1749:MT-COI (= CCOI) in colonic crypts
5154:Carbamoyl phosphate synthetase I
4794:Long-chain-fatty-acid—CoA ligase
4789:Carnitine palmitoyltransferase I
4707:
4692:
4677:
4662:
4647:
4632:
4617:
4602:
4587:
4572:
4557:
4542:
4527:
4512:
3687:10.1111/j.1474-9726.2008.00426.x
3360:10.1111/j.1474-9726.2009.00531.x
3148:10.1111/j.1574-6968.2009.01839.x
3086:Proceedings. Biological Sciences
2711:European Journal of Biochemistry
2326:10.1128/jb.176.18.5587-5600.1994
2151:
1708:to identify animal species. The
212:
132:
5015:Glutamate aspartate transporter
2199:(D1): D158–D169. January 2017.
595:response to electrical stimulus
5132:Pyruvate dehydrogenase complex
5090:Succinyl coenzyme A synthetase
4023:Molecular and Cellular Biology
3788:11858/00-001M-0000-0028-466E-C
3718:Brown GC, Borutaite V (2008).
3549:Journal of Cellular Physiology
965:Cytochrome c oxidase subunit I
534:integral component of membrane
443:More reference expression data
1:
3647:10.1016/S0021-9258(18)62305-1
2254:10.1161/CIRCRESAHA.113.301151
2012:10.1126/science.272.5265.1136
1103:Available protein structures:
498:cytochrome-c oxidase activity
4936:Dihydroorotate dehydrogenase
4182:10.1016/0022-2836(80)90196-5
4170:Journal of Molecular Biology
3828:10.1016/j.bbamcr.2017.11.010
3778:10.1016/j.celrep.2015.08.009
3737:10.1016/j.bbabio.2008.03.024
3607:10.1016/0014-4827(91)90467-9
3311:10.1016/j.celrep.2014.07.019
2605:10.1016/j.bbabio.2012.10.002
2556:10.1016/0014-5793(94)80228-9
2467:10.1016/0304-4173(83)90003-4
2079:10.1016/j.protis.2011.10.003
1438:. Some patients may display
600:response to oxidative stress
559:respiratory chain complex IV
549:mitochondrial inner membrane
4990:Steroid 11-beta-hydroxylase
4070:10.1101/sqb.1986.051.01.013
1824:MT-COI is coded for by the
1534:hypertrophic cardiomyopathy
1448:respiratory chain complexes
1270:-rich heavy (H) section of
343:stromal cell of endometrium
5601:
5532:
5164:N-Acetylglutamate synthase
5159:Ornithine transcarbamylase
4963:Glycerol phosphate shuttle
4829:monoamine neurotransmitter
3595:Experimental Cell Research
2359:Cell Biology International
1562:in the inner walls of the
1209:. The gene is also called
29:
5570:Human mitochondrial genes
5518:
5216:
5192:
4889:oxidative phosphorylation
4507:
3878:10.1016/j.tig.2006.04.001
3135:FEMS Microbiology Letters
2800:10.1007/s10048-005-0015-z
2687:10.1182/blood.V90.12.4961
2449:Capaldi RA, Malatesta F,
1973:"Mouse PubMed Reference:"
1955:"Human PubMed Reference:"
1590:, and family history are
1319:oxidative phosphorylation
1098:
969:
949:
944:
940:
933:
917:
898:
883:
864:
853:
840:
821:
810:
797:
793:
778:
774:
765:
752:
748:
733:
729:
720:
707:
703:
688:
684:
675:
660:
653:
649:
633:
610:oxidative phosphorylation
483:
479:
467:
462:
453:
440:
389:
380:
327:
318:
288:
280:
276:
259:
246:
232:
227:Mitochondrial DNA (mouse)
211:
202:
198:
181:
168:
154:
148:Mitochondrial DNA (human)
131:
122:
118:
73:
70:
60:
53:
48:
44:
39:
5080:Isocitrate dehydrogenase
4958:Malate-aspartate shuttle
4064:. 51 Pt 1 (1): 103–114.
3895:Human Molecular Genetics
2750:Human Molecular Genetics
1826:mitochondrial chromosome
1315:electron transport chain
1306:. Cytochrome c oxidase (
5127:Glutamate dehydrogenase
4914:Succinate dehydrogenase
4107:10.1126/science.3764430
3503:Journal of Cell Science
3261:10.1073/pnas.0505903103
3202:10.4251/wjgo.v2.i12.429
2915:10.1073/pnas.0811450106
2856:10.1073/pnas.0505903103
2314:Journal of Bacteriology
1594:for colorectal cancer.
1548:Colorectal cancer (CRC)
1432:optic nerve dysfunction
1198:that is encoded by the
518:oxidoreductase activity
5585:Transmembrane proteins
5523:mitochondrial diseases
5122:Aspartate transaminase
4779:fatty acid degradation
4445:10.1186/1471-2156-2-13
3561:10.1002/jcp.1041360316
3469:10.1073/pnas.072670199
3098:10.1098/rsbl.2003.0025
2763:10.1093/hmg/11.16.1797
2371:10.1006/cbir.1994.1084
2193:Nucleic Acids Research
1867:, the encoded protein
1778:
1624:non-syndromic deafness
1430:, is characterized by
1412:sensorineural deafness
1367:nitric-oxide reductase
1360:Gram-positive bacteria
1252:sensorineural deafness
1180:Cytochrome c oxidase I
1176:
605:cerebellum development
585:response to copper ion
413:superior frontal gyrus
409:Cortex of frontal lobe
5236:Mitochondrial carrier
5112:anaplerotic reactions
4948:mitochondrial shuttle
4926:pyrimidine metabolism
4269:10.1073/pnas.92.2.532
4035:10.1128/MCB.11.3.1631
3907:10.1093/hmg/1.2.140-a
3009:10.1093/jnen/64.2.123
1830:mitochondrial fission
1756:
1578:lesions, to invasive
1384:Clinical significance
1166:
911:Chr M: 0.01 – 0.01 Mb
904:Chr M: 0.01 – 0.01 Mb
405:primary visual cortex
347:right coronary artery
5243:Translocator protein
5100:Malate dehydrogenase
4995:Aldosterone synthase
3509:(Pt 13): 2653–2662.
3092:(Suppl 1): S96–S99.
2961:10.1212/wnl.55.5.644
2242:Circulation Research
1847:and D of the image.
1763:immunohistochemistry
1697:Use in DNA barcoding
1628:maternal inheritance
1518:exercise intolerance
1452:inherited maternally
1400:sideroblastic anemia
1300:cytochrome c oxidase
1240:sideroblastic anemia
1229:cytochrome c oxidase
359:smooth muscle tissue
4855:Intermembrane space
4352:2000Natur.408..708I
4260:1995PNAS...92..532H
4211:1981Natur.290..465M
4137:1985Natur.314..592C
4099:1986Sci...234..614C
3460:2002PNAS...99.5521N
3405:2002NYASA.959..434C
3252:2006PNAS..103..714G
2906:2009PNAS..106.3402N
2847:2006PNAS..103..714G
2205:10.1093/nar/gkw1099
2004:1996Sci...272.1136T
1998:(5265): 1136–1144.
1895:-dependent manner.
1530:developmental delay
620:aerobic respiration
429:ganglionic eminence
5552:, which is in the
5210:Other/to be sorted
5175:alcohol metabolism
5035:Uncoupling protein
4909:NADH dehydrogenase
3994:10.1007/bf00206061
3866:Trends in Genetics
1779:
1622:DFNM is a form of
1584:ulcerative colitis
1526:mental retardation
1444:cardiac conduction
1334:ubiquinol oxidases
1177:
755:ENSMUSG00000064351
578:Biological process
527:Cellular component
491:Molecular function
417:hippocampus proper
331:right uterine tube
5530:
5529:
5514:
5513:
5257:Mitochondrial DNA
5251:
5250:
5205:
5204:
5060:citric acid cycle
5043:
5042:
4849:
4848:
4841:Monoamine oxidase
4725:
4724:
4346:(6813): 708–713.
4205:(5806): 465–470.
4131:(6012): 592–597.
4093:(4776): 614–618.
3966:(13): 3718–3725.
3771:(10): 1644–1655.
3515:10.1242/jcs.01134
2756:(16): 1797–1805.
2680:(12): 4961–4972.
2412:(11): 2516–2525.
2320:(18): 5587–5600.
1759:intestinal glands
1612:nonsense mutation
1522:motor development
1502:liver dysfunction
1408:colorectal cancer
1395:(LHON), acquired
1317:of mitochondrial
1288:mitochondrial DNA
1248:colorectal cancer
1235:(LHON), acquired
1161:
1160:
1157:
1156:
1152:structure summary
960:
959:
956:
955:
929:
928:
894:
893:
873:
872:
849:
848:
830:
829:
806:
805:
787:
786:
761:
760:
742:
741:
716:
715:
697:
696:
645:
644:
503:metal ion binding
475:
474:
471:
470:
449:
448:
436:
435:
397:cerebellar cortex
374:
373:
272:
271:
194:
193:
16:(Redirected from
5592:
5580:Protein families
5394:
5369:
5339:
5319:
5269:
5262:
5214:
5177:
5147:
5115:
5070:Citrate synthase
5063:
5054:
5008:
4978:
4951:
4929:
4892:
4883:
4863:Adenylate kinase
4834:
4810:
4782:
4773:
4752:
4745:
4738:
4729:
4711:
4696:
4681:
4666:
4651:
4636:
4621:
4606:
4591:
4576:
4561:
4546:
4531:
4516:
4496:
4489:
4482:
4473:
4467:
4457:
4447:
4422:
4412:
4395:(6): 1475–1484.
4379:
4360:10.1038/35047064
4334:
4316:
4291:
4281:
4271:
4238:
4219:10.1038/290465a0
4193:
4164:
4145:10.1038/314592a0
4118:
4081:
4056:
4046:
4029:(3): 1631–1637.
4013:
3975:
3953:
3943:
3918:
3889:
3850:
3849:
3839:
3807:
3801:
3800:
3790:
3780:
3756:
3750:
3749:
3739:
3730:(7–8): 877–881.
3715:
3709:
3708:
3698:
3666:
3660:
3659:
3649:
3640:(8): 2425–2429.
3625:
3619:
3618:
3590:
3581:
3580:
3544:
3535:
3534:
3498:
3492:
3491:
3481:
3471:
3454:(8): 5521–5526.
3439:
3433:
3432:
3388:
3382:
3381:
3371:
3339:
3333:
3332:
3322:
3290:
3284:
3283:
3273:
3263:
3231:
3225:
3224:
3214:
3204:
3180:
3161:
3160:
3150:
3126:
3120:
3119:
3109:
3077:
3071:
3070:
3060:
3043:(6): 1803–1806.
3028:
3022:
3021:
3011:
2987:
2981:
2980:
2944:
2938:
2937:
2927:
2917:
2900:(9): 3402–3407.
2885:
2879:
2878:
2868:
2858:
2826:
2820:
2819:
2782:
2776:
2775:
2765:
2741:
2735:
2734:
2706:
2700:
2699:
2689:
2665:
2659:
2658:
2648:
2624:
2618:
2617:
2607:
2583:
2577:
2576:
2558:
2534:
2528:
2527:
2517:
2500:(9): 2819–2823.
2494:The EMBO Journal
2485:
2479:
2478:
2446:
2440:
2439:
2429:
2406:The EMBO Journal
2397:
2391:
2390:
2354:
2348:
2347:
2337:
2305:
2294:
2293:
2282:
2276:
2275:
2265:
2248:(9): 1043–1053.
2233:
2227:
2226:
2216:
2185:
2162:
2155:
2149:
2147:
2146:
2132:
2109:
2100:
2091:
2090:
2064:
2055:
2049:
2048:
2041:
2032:
2031:
1987:
1981:
1980:
1969:
1963:
1962:
1951:
1945:
1935:
1924:
1914:
1757:Colonic crypts (
1582:. Long-standing
1414:, and recurrent
1302:, also known as
1254:, and recurrent
1186:) also known as
1167:Location of the
1100:
974:
962:
942:
941:
913:
906:
889:
877:
868:
858:
854:RefSeq (protein)
844:
834:
825:
815:
791:
772:
746:
727:
701:
682:
651:
481:
460:
445:
385:
383:Top expressed in
378:
323:
321:Top expressed in
316:
295:
278:
268:
255:
244:
228:
216:
200:
190:
177:
166:
150:
136:
120:
114:
112:COX1 - orthologs
65:
58:
37:
32:cyclooxygenase-1
21:
5600:
5599:
5595:
5594:
5593:
5591:
5590:
5589:
5575:Protein domains
5560:
5559:
5545:
5531:
5526:
5510:
5390:
5385:
5365:
5360:
5335:
5330:
5315:
5310:
5265:
5247:
5201:
5188:
5173:
5168:
5141:
5136:
5109:
5104:
5057:
5039:
5004:
4999:
4975:steroidogenesis
4972:
4967:
4945:
4940:
4923:
4918:
4886:
4872:
4868:Creatine kinase
4845:
4831:
4826:
4821:
4803:
4798:
4776:
4762:
4756:
4726:
4721:
4718:
4712:
4703:
4697:
4688:
4682:
4673:
4667:
4658:
4652:
4643:
4637:
4628:
4622:
4613:
4607:
4598:
4592:
4583:
4577:
4568:
4562:
4553:
4547:
4538:
4532:
4523:
4517:
4503:
4500:
4470:
4425:
4382:
4337:
4301:Nature Genetics
4294:
4241:
4196:
4167:
4121:
4084:
4059:
4016:
3978:
3960:Cancer Research
3956:
3921:
3892:
3863:
3859:
3857:Further reading
3854:
3853:
3809:
3808:
3804:
3758:
3757:
3753:
3717:
3716:
3712:
3668:
3667:
3663:
3627:
3626:
3622:
3592:
3591:
3584:
3546:
3545:
3538:
3500:
3499:
3495:
3441:
3440:
3436:
3390:
3389:
3385:
3341:
3340:
3336:
3292:
3291:
3287:
3233:
3232:
3228:
3195:(12): 429–442.
3182:
3181:
3164:
3128:
3127:
3123:
3079:
3078:
3074:
3030:
3029:
3025:
2989:
2988:
2984:
2946:
2945:
2941:
2887:
2886:
2882:
2828:
2827:
2823:
2784:
2783:
2779:
2743:
2742:
2738:
2708:
2707:
2703:
2667:
2666:
2662:
2626:
2625:
2621:
2585:
2584:
2580:
2536:
2535:
2531:
2487:
2486:
2482:
2451:Darley-Usmar VM
2448:
2447:
2443:
2399:
2398:
2394:
2356:
2355:
2351:
2307:
2306:
2297:
2284:
2283:
2279:
2235:
2234:
2230:
2187:
2186:
2165:
2144:
2142:
2140:www.uniprot.org
2134:
2133:
2112:
2101:
2094:
2062:
2057:
2056:
2052:
2043:
2042:
2035:
1989:
1988:
1984:
1971:
1970:
1966:
1953:
1952:
1948:
1936:
1927:
1915:
1906:
1901:
1861:
1791:large intestine
1751:
1699:
1636:
1620:
1600:
1550:
1514:muscle weakness
1494:
1460:
1424:
1386:
1284:
1264:
1064:OPM superfamily
977:
951:View/Edit Mouse
946:View/Edit Human
909:
902:
899:Location (UCSC)
885:
866:
842:
823:
736:ENSG00000198804
629:
573:
522:
508:protein binding
441:
432:
427:
423:
421:muscle of thigh
419:
415:
411:
407:
403:
399:
395:
381:
370:
365:
361:
357:
355:Brodmann area 9
353:
349:
345:
341:
337:
333:
319:
263:
250:
242:
226:
203:Gene location (
185:
172:
164:
146:
123:Gene location (
74:
61:
54:
35:
28:
23:
22:
15:
12:
11:
5:
5598:
5596:
5588:
5587:
5582:
5577:
5572:
5562:
5561:
5528:
5527:
5519:
5516:
5515:
5512:
5511:
5509:
5508:
5503:
5498:
5493:
5488:
5483:
5478:
5473:
5468:
5463:
5458:
5453:
5448:
5443:
5438:
5433:
5428:
5423:
5418:
5413:
5408:
5403:
5397:
5395:
5387:
5386:
5384:
5383:
5378:
5372:
5370:
5362:
5361:
5359:
5358:
5353:
5348:
5342:
5340:
5332:
5331:
5329:
5328:
5322:
5320:
5312:
5311:
5309:
5308:
5303:
5298:
5293:
5288:
5283:
5278:
5272:
5270:
5259:
5253:
5252:
5249:
5248:
5246:
5245:
5240:
5239:
5238:
5233:
5223:
5217:
5211:
5207:
5206:
5203:
5202:
5200:
5199:
5193:
5190:
5189:
5187:
5186:
5180:
5178:
5170:
5169:
5167:
5166:
5161:
5156:
5150:
5148:
5138:
5137:
5135:
5134:
5129:
5124:
5118:
5116:
5106:
5105:
5103:
5102:
5097:
5092:
5087:
5082:
5077:
5072:
5066:
5064:
5051:
5045:
5044:
5041:
5040:
5038:
5037:
5032:
5027:
5022:
5017:
5011:
5009:
5001:
5000:
4998:
4997:
4992:
4987:
4981:
4979:
4969:
4968:
4966:
4965:
4960:
4954:
4952:
4942:
4941:
4939:
4938:
4932:
4930:
4920:
4919:
4917:
4916:
4911:
4906:
4901:
4895:
4893:
4880:
4878:Inner membrane
4874:
4873:
4871:
4870:
4865:
4859:
4857:
4851:
4850:
4847:
4846:
4844:
4843:
4837:
4835:
4823:
4822:
4820:
4819:
4813:
4811:
4800:
4799:
4797:
4796:
4791:
4785:
4783:
4770:
4768:Outer membrane
4764:
4763:
4757:
4755:
4754:
4747:
4740:
4732:
4723:
4722:
4720:
4719:
4713:
4706:
4704:
4698:
4691:
4689:
4683:
4676:
4674:
4668:
4661:
4659:
4653:
4646:
4644:
4638:
4631:
4629:
4623:
4616:
4614:
4608:
4601:
4599:
4593:
4586:
4584:
4578:
4571:
4569:
4563:
4556:
4554:
4548:
4541:
4539:
4533:
4526:
4524:
4518:
4511:
4508:
4505:
4504:
4501:
4499:
4498:
4491:
4484:
4476:
4469:
4468:
4423:
4401:10.1086/320591
4380:
4335:
4292:
4254:(2): 532–536.
4239:
4194:
4176:(2): 161–178.
4165:
4119:
4082:
4057:
4014:
3988:(2): 139–145.
3982:Human Genetics
3976:
3954:
3934:(2): 378–385.
3919:
3890:
3872:(6): 339–345.
3860:
3858:
3855:
3852:
3851:
3822:(2): 323–333.
3802:
3751:
3710:
3681:(6): 920–923.
3661:
3620:
3601:(1): 137–140.
3582:
3555:(3): 507–513.
3536:
3493:
3434:
3399:(1): 434–447.
3383:
3334:
3305:(4): 940–947.
3285:
3246:(3): 714–719.
3226:
3162:
3121:
3072:
3049:10.1086/302658
3023:
3002:(2): 123–128.
2982:
2955:(5): 644–649.
2939:
2880:
2841:(3): 714–719.
2821:
2777:
2736:
2717:(1): 132–138.
2701:
2660:
2639:(2): 378–385.
2619:
2598:(2): 136–144.
2578:
2529:
2480:
2461:(2): 135–148.
2441:
2392:
2365:(5): 345–355.
2349:
2295:
2277:
2228:
2163:
2110:
2092:
2073:(3): 415–434.
2050:
2033:
1982:
1964:
1946:
1925:
1903:
1902:
1900:
1897:
1860:
1857:
1837:selection bias
1787:colonic crypts
1750:
1747:
1698:
1695:
1694:
1693:
1679:
1665:
1651:
1635:
1632:
1619:
1616:
1604:rhabdomyolysis
1599:
1596:
1580:adenocarcinoma
1549:
1546:
1493:
1490:
1459:
1456:
1423:
1420:
1385:
1382:
1356:Pseudomonadota
1283:
1280:
1263:
1260:
1159:
1158:
1155:
1154:
1149:
1143:
1142:
1129:
1123:
1122:
1112:
1105:
1104:
1096:
1095:
1090:
1084:
1083:
1078:
1072:
1071:
1066:
1060:
1059:
1054:
1048:
1047:
1034:
1028:
1027:
1022:
1016:
1015:
1010:
1004:
1003:
998:
992:
991:
988:
984:
983:
979:
978:
975:
967:
966:
958:
957:
954:
953:
948:
938:
937:
931:
930:
927:
926:
924:
922:
915:
914:
907:
900:
896:
895:
892:
891:
881:
880:
874:
871:
870:
862:
861:
855:
851:
850:
847:
846:
838:
837:
831:
828:
827:
819:
818:
812:
808:
807:
804:
803:
795:
794:
788:
785:
784:
776:
775:
769:
763:
762:
759:
758:
750:
749:
743:
740:
739:
731:
730:
724:
718:
717:
714:
713:
705:
704:
698:
695:
694:
686:
685:
679:
673:
672:
667:
662:
658:
657:
647:
646:
643:
642:
631:
630:
628:
627:
622:
617:
612:
607:
602:
597:
592:
587:
581:
579:
575:
574:
572:
571:
566:
561:
556:
551:
546:
541:
536:
530:
528:
524:
523:
521:
520:
515:
510:
505:
500:
494:
492:
488:
487:
477:
476:
473:
472:
469:
468:
465:
464:
457:
451:
450:
447:
446:
438:
437:
434:
433:
431:
430:
426:
422:
418:
414:
410:
406:
402:
398:
394:
390:
387:
386:
375:
372:
371:
369:
368:
364:
360:
356:
352:
348:
344:
340:
336:
332:
328:
325:
324:
312:
311:
303:
292:
286:
285:
282:RNA expression
274:
273:
270:
269:
261:
257:
256:
248:
245:
240:
234:
233:
230:
229:
224:
218:
217:
209:
208:
196:
195:
192:
191:
183:
179:
178:
170:
167:
162:
156:
155:
152:
151:
144:
138:
137:
129:
128:
116:
115:
72:
68:
67:
59:
51:
50:
46:
45:
42:
41:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
5597:
5586:
5583:
5581:
5578:
5576:
5573:
5571:
5568:
5567:
5565:
5558:
5557:
5555:
5554:public domain
5551:
5544:
5540:
5536:
5525:
5524:
5517:
5507:
5504:
5502:
5499:
5497:
5494:
5492:
5489:
5487:
5484:
5482:
5479:
5477:
5474:
5472:
5469:
5467:
5464:
5462:
5459:
5457:
5454:
5452:
5449:
5447:
5444:
5442:
5439:
5437:
5434:
5432:
5429:
5427:
5424:
5422:
5419:
5417:
5414:
5412:
5409:
5407:
5404:
5402:
5399:
5398:
5396:
5393:
5388:
5382:
5379:
5377:
5374:
5373:
5371:
5368:
5363:
5357:
5354:
5352:
5349:
5347:
5344:
5343:
5341:
5338:
5333:
5327:
5324:
5323:
5321:
5318:
5313:
5307:
5304:
5302:
5299:
5297:
5294:
5292:
5289:
5287:
5284:
5282:
5279:
5277:
5274:
5273:
5271:
5268:
5263:
5260:
5258:
5254:
5244:
5241:
5237:
5234:
5232:
5229:
5228:
5227:
5224:
5222:
5219:
5218:
5215:
5212:
5208:
5198:
5195:
5194:
5191:
5185:
5182:
5181:
5179:
5176:
5171:
5165:
5162:
5160:
5157:
5155:
5152:
5151:
5149:
5146:
5145:
5139:
5133:
5130:
5128:
5125:
5123:
5120:
5119:
5117:
5114:
5113:
5107:
5101:
5098:
5096:
5093:
5091:
5088:
5086:
5083:
5081:
5078:
5076:
5073:
5071:
5068:
5067:
5065:
5062:
5061:
5055:
5052:
5050:
5046:
5036:
5033:
5031:
5028:
5026:
5023:
5021:
5018:
5016:
5013:
5012:
5010:
5007:
5002:
4996:
4993:
4991:
4988:
4986:
4983:
4982:
4980:
4977:
4976:
4970:
4964:
4961:
4959:
4956:
4955:
4953:
4950:
4949:
4943:
4937:
4934:
4933:
4931:
4928:
4927:
4921:
4915:
4912:
4910:
4907:
4905:
4902:
4900:
4897:
4896:
4894:
4891:
4890:
4884:
4881:
4879:
4875:
4869:
4866:
4864:
4861:
4860:
4858:
4856:
4852:
4842:
4839:
4838:
4836:
4833:
4830:
4824:
4818:
4815:
4814:
4812:
4809:
4807:
4801:
4795:
4792:
4790:
4787:
4786:
4784:
4781:
4780:
4774:
4771:
4769:
4765:
4760:
4759:Mitochondrial
4753:
4748:
4746:
4741:
4739:
4734:
4733:
4730:
4716:
4710:
4705:
4701:
4695:
4690:
4686:
4680:
4675:
4671:
4665:
4660:
4656:
4650:
4645:
4641:
4635:
4630:
4626:
4620:
4615:
4611:
4605:
4600:
4596:
4590:
4585:
4581:
4575:
4570:
4566:
4560:
4555:
4551:
4545:
4540:
4536:
4530:
4525:
4521:
4515:
4510:
4506:
4497:
4492:
4490:
4485:
4483:
4478:
4477:
4474:
4465:
4461:
4456:
4451:
4446:
4441:
4437:
4433:
4429:
4424:
4420:
4416:
4411:
4406:
4402:
4398:
4394:
4390:
4386:
4381:
4377:
4373:
4369:
4365:
4361:
4357:
4353:
4349:
4345:
4341:
4336:
4332:
4328:
4324:
4320:
4315:
4314:10.1038/13779
4310:
4306:
4302:
4298:
4293:
4289:
4285:
4280:
4275:
4270:
4265:
4261:
4257:
4253:
4249:
4245:
4240:
4236:
4232:
4228:
4224:
4220:
4216:
4212:
4208:
4204:
4200:
4195:
4191:
4187:
4183:
4179:
4175:
4171:
4166:
4162:
4158:
4154:
4150:
4146:
4142:
4138:
4134:
4130:
4126:
4120:
4116:
4112:
4108:
4104:
4100:
4096:
4092:
4088:
4083:
4079:
4075:
4071:
4067:
4063:
4058:
4054:
4050:
4045:
4040:
4036:
4032:
4028:
4024:
4020:
4015:
4011:
4007:
4003:
3999:
3995:
3991:
3987:
3983:
3977:
3973:
3969:
3965:
3961:
3955:
3951:
3947:
3942:
3937:
3933:
3929:
3925:
3920:
3916:
3912:
3908:
3904:
3900:
3896:
3891:
3887:
3883:
3879:
3875:
3871:
3867:
3862:
3861:
3856:
3847:
3843:
3838:
3833:
3829:
3825:
3821:
3817:
3813:
3806:
3803:
3798:
3794:
3789:
3784:
3779:
3774:
3770:
3766:
3762:
3755:
3752:
3747:
3743:
3738:
3733:
3729:
3725:
3721:
3714:
3711:
3706:
3702:
3697:
3692:
3688:
3684:
3680:
3676:
3672:
3665:
3662:
3657:
3653:
3648:
3643:
3639:
3635:
3631:
3624:
3621:
3616:
3612:
3608:
3604:
3600:
3596:
3589:
3587:
3583:
3578:
3574:
3570:
3566:
3562:
3558:
3554:
3550:
3543:
3541:
3537:
3532:
3528:
3524:
3520:
3516:
3512:
3508:
3504:
3497:
3494:
3489:
3485:
3480:
3475:
3470:
3465:
3461:
3457:
3453:
3449:
3445:
3438:
3435:
3430:
3426:
3422:
3418:
3414:
3410:
3406:
3402:
3398:
3394:
3387:
3384:
3379:
3375:
3370:
3365:
3361:
3357:
3353:
3349:
3345:
3338:
3335:
3330:
3326:
3321:
3316:
3312:
3308:
3304:
3300:
3296:
3289:
3286:
3281:
3277:
3272:
3267:
3262:
3257:
3253:
3249:
3245:
3241:
3237:
3230:
3227:
3222:
3218:
3213:
3208:
3203:
3198:
3194:
3190:
3186:
3179:
3177:
3175:
3173:
3171:
3169:
3167:
3163:
3158:
3154:
3149:
3144:
3140:
3136:
3132:
3125:
3122:
3117:
3113:
3108:
3103:
3099:
3095:
3091:
3087:
3083:
3076:
3073:
3068:
3064:
3059:
3054:
3050:
3046:
3042:
3038:
3034:
3027:
3024:
3019:
3015:
3010:
3005:
3001:
2997:
2993:
2986:
2983:
2978:
2974:
2970:
2966:
2962:
2958:
2954:
2950:
2943:
2940:
2935:
2931:
2926:
2921:
2916:
2911:
2907:
2903:
2899:
2895:
2891:
2884:
2881:
2876:
2872:
2867:
2862:
2857:
2852:
2848:
2844:
2840:
2836:
2832:
2825:
2822:
2817:
2813:
2809:
2805:
2801:
2797:
2793:
2789:
2788:Neurogenetics
2781:
2778:
2773:
2769:
2764:
2759:
2755:
2751:
2747:
2740:
2737:
2732:
2728:
2724:
2720:
2716:
2712:
2705:
2702:
2697:
2693:
2688:
2683:
2679:
2675:
2671:
2664:
2661:
2656:
2652:
2647:
2642:
2638:
2634:
2630:
2623:
2620:
2615:
2611:
2606:
2601:
2597:
2593:
2589:
2582:
2579:
2574:
2570:
2566:
2562:
2557:
2552:
2548:
2544:
2540:
2533:
2530:
2525:
2521:
2516:
2511:
2507:
2503:
2499:
2495:
2491:
2484:
2481:
2476:
2472:
2468:
2464:
2460:
2456:
2452:
2445:
2442:
2437:
2433:
2428:
2423:
2419:
2415:
2411:
2407:
2403:
2396:
2393:
2388:
2384:
2380:
2376:
2372:
2368:
2364:
2360:
2353:
2350:
2345:
2341:
2336:
2331:
2327:
2323:
2319:
2315:
2311:
2304:
2302:
2300:
2296:
2291:
2287:
2281:
2278:
2273:
2269:
2264:
2259:
2255:
2251:
2247:
2243:
2239:
2232:
2229:
2224:
2220:
2215:
2210:
2206:
2202:
2198:
2194:
2190:
2184:
2182:
2180:
2178:
2176:
2174:
2172:
2170:
2168:
2164:
2161:
2159:
2154:
2141:
2137:
2131:
2129:
2127:
2125:
2123:
2121:
2119:
2117:
2115:
2111:
2108:
2104:
2099:
2097:
2093:
2088:
2084:
2080:
2076:
2072:
2068:
2061:
2054:
2051:
2046:
2040:
2038:
2034:
2029:
2025:
2021:
2017:
2013:
2009:
2005:
2001:
1997:
1993:
1986:
1983:
1978:
1974:
1968:
1965:
1960:
1956:
1950:
1947:
1943:
1939:
1934:
1932:
1930:
1926:
1922:
1918:
1913:
1911:
1909:
1905:
1898:
1896:
1894:
1890:
1886:
1882:
1878:
1874:
1870:
1866:
1858:
1856:
1852:
1848:
1844:
1842:
1838:
1833:
1831:
1827:
1822:
1818:
1814:
1810:
1806:
1804:
1799:
1794:
1792:
1789:of the human
1788:
1784:
1776:
1772:
1768:
1767:mitochondrial
1764:
1760:
1755:
1748:
1746:
1744:
1740:
1736:
1732:
1728:
1723:
1719:
1715:
1711:
1707:
1703:
1696:
1692:
1691:
1686:
1683:
1680:
1678:
1677:
1672:
1669:
1666:
1664:
1663:
1658:
1655:
1652:
1650:
1649:
1644:
1641:
1638:
1637:
1633:
1631:
1629:
1625:
1617:
1615:
1613:
1609:
1605:
1597:
1595:
1593:
1589:
1585:
1581:
1577:
1573:
1569:
1565:
1561:
1558:
1554:
1547:
1545:
1543:
1542:Leigh disease
1539:
1535:
1531:
1527:
1523:
1519:
1515:
1511:
1507:
1503:
1499:
1491:
1489:
1487:
1483:
1479:
1475:
1472:
1468:
1464:
1457:
1455:
1453:
1449:
1445:
1441:
1437:
1433:
1429:
1419:
1417:
1416:myoglobinuria
1413:
1409:
1405:
1401:
1398:
1394:
1390:
1383:
1381:
1379:
1375:
1372:
1368:
1363:
1361:
1357:
1353:
1348:
1344:
1342:
1339:
1335:
1331:
1327:
1322:
1320:
1316:
1312:
1309:
1305:
1301:
1297:
1293:
1289:
1281:
1279:
1277:
1273:
1269:
1261:
1259:
1257:
1256:myoglobinuria
1253:
1249:
1245:
1241:
1238:
1234:
1230:
1226:
1225:
1220:
1219:
1214:
1213:
1208:
1204:
1201:
1197:
1193:
1189:
1185:
1181:
1174:
1170:
1165:
1153:
1150:
1148:
1144:
1141:
1137:
1133:
1130:
1128:
1124:
1120:
1116:
1113:
1110:
1106:
1101:
1097:
1094:
1091:
1089:
1085:
1082:
1079:
1077:
1073:
1070:
1067:
1065:
1061:
1058:
1055:
1053:
1049:
1046:
1042:
1038:
1035:
1033:
1029:
1026:
1023:
1021:
1017:
1014:
1011:
1009:
1005:
1002:
999:
997:
993:
989:
985:
980:
973:
968:
963:
952:
947:
943:
939:
936:
932:
925:
923:
920:
916:
912:
908:
905:
901:
897:
890:
888:
882:
878:
875:
869:
863:
859:
856:
852:
845:
839:
835:
832:
826:
820:
816:
813:
811:RefSeq (mRNA)
809:
802:
801:
796:
792:
789:
783:
782:
777:
773:
770:
768:
764:
757:
756:
751:
747:
744:
738:
737:
732:
728:
725:
723:
719:
712:
711:
706:
702:
699:
693:
692:
687:
683:
680:
678:
674:
671:
668:
666:
663:
659:
656:
652:
648:
641:
637:
632:
626:
623:
621:
618:
616:
613:
611:
608:
606:
603:
601:
598:
596:
593:
591:
588:
586:
583:
582:
580:
577:
576:
570:
567:
565:
562:
560:
557:
555:
552:
550:
547:
545:
544:mitochondrion
542:
540:
537:
535:
532:
531:
529:
526:
525:
519:
516:
514:
511:
509:
506:
504:
501:
499:
496:
495:
493:
490:
489:
486:
485:Gene ontology
482:
478:
466:
461:
458:
456:
452:
444:
439:
428:
424:
420:
416:
412:
408:
404:
400:
396:
392:
391:
388:
384:
379:
376:
366:
362:
358:
354:
350:
346:
342:
338:
335:apex of heart
334:
330:
329:
326:
322:
317:
314:
313:
310:
308:
304:
302:
301:
297:
296:
293:
291:
287:
283:
279:
275:
267:
262:
258:
254:
249:
239:
235:
231:
223:
219:
215:
210:
206:
201:
197:
189:
184:
180:
176:
171:
161:
157:
153:
149:
143:
139:
135:
130:
126:
121:
117:
113:
109:
105:
101:
97:
93:
89:
85:
81:
77:
69:
64:
57:
52:
47:
43:
38:
33:
19:
5547:
5546:
5520:
5367:ATP synthase
5345:
5174:
5142:
5110:
5058:
5025:ATP synthase
5005:
4973:
4946:
4924:
4904:Cytochrome c
4887:
4827:
4817:Kynureninase
4804:
4777:
4714:
4699:
4684:
4669:
4654:
4639:
4624:
4609:
4594:
4579:
4564:
4549:
4534:
4519:
4435:
4432:BMC Genetics
4431:
4392:
4388:
4343:
4339:
4304:
4300:
4251:
4247:
4202:
4198:
4173:
4169:
4128:
4124:
4090:
4086:
4061:
4026:
4022:
3985:
3981:
3963:
3959:
3931:
3927:
3898:
3894:
3869:
3865:
3819:
3815:
3805:
3768:
3765:Cell Reports
3764:
3754:
3727:
3723:
3713:
3678:
3674:
3664:
3637:
3633:
3623:
3598:
3594:
3552:
3548:
3506:
3502:
3496:
3451:
3447:
3437:
3396:
3392:
3386:
3354:(1): 96–99.
3351:
3347:
3337:
3302:
3299:Cell Reports
3298:
3288:
3243:
3239:
3229:
3192:
3188:
3141:(1): 76–84.
3138:
3134:
3124:
3089:
3085:
3075:
3040:
3036:
3026:
2999:
2995:
2985:
2952:
2948:
2942:
2897:
2893:
2883:
2838:
2834:
2824:
2794:(1): 51–57.
2791:
2787:
2780:
2753:
2749:
2739:
2714:
2710:
2704:
2677:
2673:
2663:
2636:
2632:
2622:
2595:
2591:
2581:
2546:
2543:FEBS Letters
2542:
2532:
2497:
2493:
2483:
2458:
2454:
2444:
2409:
2405:
2395:
2362:
2358:
2352:
2317:
2313:
2289:
2280:
2245:
2241:
2231:
2196:
2192:
2150:
2143:. Retrieved
2139:
2070:
2066:
2053:
1995:
1991:
1985:
1976:
1967:
1958:
1949:
1862:
1859:Interactions
1853:
1849:
1845:
1834:
1823:
1819:
1815:
1811:
1807:
1795:
1780:
1775:haematoxylin
1734:
1730:
1721:
1709:
1701:
1700:
1688:
1674:
1660:
1646:
1621:
1601:
1592:risk factors
1588:colon polyps
1576:premalignant
1552:
1551:
1506:hepatomegaly
1495:
1482:biosynthesis
1462:
1461:
1440:neurological
1427:
1425:
1406:deficiency,
1387:
1377:
1364:
1351:
1349:
1345:
1323:
1285:
1265:
1246:deficiency,
1223:
1222:
1217:
1216:
1211:
1210:
1199:
1191:
1187:
1183:
1179:
1178:
1172:
1168:
884:
865:
841:
822:
798:
779:
753:
734:
708:
689:
669:
664:
590:human ageing
513:heme binding
305:
298:
71:External IDs
5317:Complex III
4502:PDB gallery
1727:seed plants
1706:DNA barcode
1634:Subfamilies
1436:vision loss
1326:prokaryotes
1276:amino acids
1076:OPM protein
990:COX1 or COI
982:Identifiers
554:respirasome
363:gallbladder
49:Identifiers
5564:Categories
5337:Complex IV
5144:urea cycle
4832:metabolism
4808:metabolism
4806:tryptophan
4307:(2): 147.
3901:(2): 140.
3675:Aging Cell
3348:Aging Cell
2549:(1): 1–4.
2145:2018-08-13
1944:, May 2017
1923:, May 2017
1899:References
1841:homoplasmy
1798:epithelial
1739:soil fungi
1520:, delayed
1404:Complex IV
1397:idiopathic
1365:A related
1330:eukaryotes
1304:complex IV
1244:Complex IV
1237:idiopathic
1207:eukaryotes
1115:structures
309:(ortholog)
92:HomoloGene
5543:IPR000883
5521:see also
5267:Complex I
5075:Aconitase
2949:Neurology
2158:CC BY 4.0
1869:interacts
1783:cytoplasm
1690:IPR014241
1676:IPR014233
1662:IPR014207
1648:IPR004677
1608:myoglobin
1557:malignant
1510:hypotonia
1467:reduction
1389:Mutations
1341:1.10.3.10
1262:Structure
1025:PDOC00074
1013:IPR000883
887:NP_904330
655:Orthologs
401:esophagus
100:GeneCards
5539:InterPro
5221:Frataxin
5095:Fumarase
4761:proteins
4464:11553319
4419:11349229
4376:52850476
4368:11130070
4331:32212178
4323:10508508
4161:32964006
4010:28048453
3886:16678300
3846:29154948
3797:26321642
3746:18439415
3705:18691181
3531:14335558
3523:15138283
3488:11943860
3429:40639679
3421:11976216
3378:19878146
3329:25127143
3280:16407113
3221:21191537
3157:19909345
3116:12952648
3067:10577941
3018:15751226
2977:26776388
2969:10980727
2934:19218458
2875:16407113
2816:21304246
2808:16284789
2772:12140182
2614:23044391
2387:36428993
2272:23965338
2223:27899622
2160:license.
2105:(OMIM):
2087:22130576
2028:20860573
1940:–
1919:–
1885:turnover
1714:mutation
1685:InterPro
1671:InterPro
1657:InterPro
1643:InterPro
1538:neonatal
1498:myopathy
1450:, it is
1374:1.7.99.7
1282:Function
1132:RCSB PDB
1008:InterPro
935:Wikidata
634:Sources:
539:membrane
367:appendix
339:duodenum
5381:MT-ATP8
5376:MT-ATP6
5296:MT-ND4L
4410:1226134
4348:Bibcode
4288:7530363
4256:Bibcode
4235:4358928
4227:7219535
4207:Bibcode
4190:6260957
4153:3921850
4133:Bibcode
4115:3764430
4095:Bibcode
4087:Science
4078:3472707
4053:1996112
4002:1757091
3972:1377597
3950:1322638
3941:1682694
3915:1301157
3837:5764226
3696:2659384
3656:5553400
3615:1715276
3577:2841036
3569:3170646
3456:Bibcode
3401:Bibcode
3369:2816353
3320:4471679
3271:1325106
3248:Bibcode
3212:3011097
3107:1698023
3058:1288397
2925:2651238
2902:Bibcode
2866:1325106
2843:Bibcode
2731:9851701
2696:9389715
2655:1322638
2646:1682694
2573:1248917
2565:8137905
2524:2824194
2475:6307356
2436:8013452
2379:8049679
2344:8083153
2263:4076475
2214:5210571
2067:Protist
2020:8638158
2000:Bibcode
1992:Science
1942:Ensembl
1921:Ensembl
1889:TMEM177
1881:MITRAC7
1865:complex
1718:species
1687::
1673::
1659::
1645::
1572:adenoma
1560:lesions
1478:ferrous
1311:1.9.3.1
1268:guanine
1196:protein
1194:) is a
1093:cd01663
1020:PROSITE
1001:PF00115
767:UniProt
722:Ensembl
661:Species
640:QuickGO
425:jejunum
284:pattern
56:Aliases
5486:MT-TS2
5481:MT-TS1
5451:MT-TL2
5446:MT-TL1
5356:MT-CO3
5351:MT-CO2
5346:MT-CO1
5326:MT-CYB
5306:MT-ND6
5301:MT-ND5
5291:MT-ND4
5286:MT-ND3
5281:MT-ND2
5276:MT-ND1
5049:Matrix
4462:
4452:
4438:: 13.
4417:
4407:
4374:
4366:
4340:Nature
4329:
4321:
4286:
4276:
4233:
4225:
4199:Nature
4188:
4159:
4151:
4125:Nature
4113:
4076:
4051:
4044:369459
4041:
4008:
4000:
3970:
3948:
3938:
3913:
3884:
3844:
3834:
3795:
3744:
3703:
3693:
3654:
3613:
3575:
3567:
3529:
3521:
3486:
3479:122802
3476:
3427:
3419:
3376:
3366:
3327:
3317:
3278:
3268:
3219:
3209:
3155:
3114:
3104:
3065:
3055:
3016:
2975:
2967:
2932:
2922:
2873:
2863:
2814:
2806:
2770:
2729:
2694:
2653:
2643:
2612:
2571:
2563:
2522:
2515:553708
2512:
2473:
2434:
2427:395125
2424:
2385:
2377:
2342:
2335:196760
2332:
2270:
2260:
2221:
2211:
2107:516030
2085:
2026:
2018:
1877:SMIM20
1771:nuclei
1735:MT-CO1
1731:MT-CO1
1722:MT-CO1
1710:MT-CO1
1702:MT-CO1
1568:rectum
1553:MT-CO1
1532:, and
1471:ferric
1463:MT-CO1
1428:MT-CO1
1296:MT-CO3
1292:MT-CO2
1200:MT-CO1
1192:MT-CO1
1173:MT-CO1
1169:MT-CO1
1147:PDBsum
1121:
1111:
1045:SUPFAM
987:Symbol
921:search
919:PubMed
800:P00397
781:P00395
677:Entrez
455:BioGPS
351:rectum
264:6,872
251:5,328
186:7,445
173:5,904
88:102504
80:516030
5506:MT-TY
5501:MT-TW
5496:MT-TV
5491:MT-TT
5476:MT-TR
5471:MT-TQ
5466:MT-TP
5461:MT-TN
5456:MT-TM
5441:MT-TK
5436:MT-TI
5431:MT-TH
5426:MT-TG
5421:MT-TF
5416:MT-TE
5411:MT-TD
5406:MT-TC
5401:MT-TA
5197:PMPCB
5184:ALDH2
5006:other
4455:55343
4372:S2CID
4327:S2CID
4279:42775
4231:S2CID
4157:S2CID
4006:S2CID
3573:S2CID
3527:S2CID
3425:S2CID
2973:S2CID
2812:S2CID
2674:Blood
2569:S2CID
2383:S2CID
2063:(PDF)
2024:S2CID
1893:COX20
1891:in a
1871:with
1803:lumen
1741:than
1626:with
1574:, or
1564:colon
1298:) of
1272:mtDNA
1221:, or
1057:3.D.4
1041:SCOPe
1032:SCOP2
710:17708
670:Mouse
665:Human
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