1664:
584:
4448:
29:
1041:
1480:
1117:
1124:
The reaction mechanism for complex III (cytochrome bc1, coenzyme Q: cytochrome C oxidoreductase) is known as the ubiquinone ("Q") cycle. In this cycle four protons get released into the positive "P" side (inter membrane space), but only two protons get taken up from the negative "N" side (matrix). As
1697:
Gao X, Wen X, Esser L, Quinn B, Yu L, Yu CA, Xia D (August 2003). "Structural basis for the quinone reduction in the bc1 complex: a comparative analysis of crystal structures of mitochondrial cytochrome bc1 with bound substrate and inhibitors at the Qi site".
1628:, which in neonates are lethal conditions that have multisystem and neurologic manifestations typifying severe mitochondrial disorders. The pathogenicity of several mutations has been verified in model systems such as yeast.
1445:
produced at the Qo site can be released both into the mitochondrial matrix and into the intermembrane space, where it can then reach the cytosol. This could be explained by the fact that
Complex III might produce
3119:
2391:
Schuelke M, Krude H, Finckh B, Mayatepek E, Janssen A, Schmelz M, Trefz F, Trijbels F, Smeitink J (March 2002). "Septo-optic dysplasia associated with a new mitochondrial cytochrome b mutation".
1603:: Newly identified subunit, mutations linked to complex III deficiency nuclear type 2. Helps remove the N-terminal fragment of UQCRFS1, which would otherwise interfere with complex III function.
2743:
4025:
685:
complex, or
Complex III, contains 11 subunits: 3 respiratory subunits, 2 core proteins and 6 low-molecular weight proteins. Proteobacterial complexes may contain as few as three subunits.
1504:
1733:
Iwata S, Lee JW, Okada K, Lee JK, Iwata M, Rasmussen B, Link TA, Ramaswamy S, Jap BK (July 1998). "Complete structure of the 11-subunit bovine mitochondrial cytochrome bc1 complex".
595:, the number of subunits found can be small, as small as three polypeptide chains. This number does increase, and eleven subunits are found in higher animals. Three subunits have
427:
2434:
Wibrand F, Ravn K, Schwartz M, Rosenberg T, Horn N, Vissing J (October 2001). "Multisystem disorder associated with a missense mutation in the mitochondrial cytochrome b gene".
3220:
3112:
3082:
288:
2736:
2690:
100:
88:
4018:
3016:
2257:
Bottani, E; Cerutti, R; Harbour, ME; Ravaglia, S; Dogan, SA; Giordano, C; Fearnley, IM; D'Amati, G; Viscomi, C; Fernandez-Vizarra, E; Zeviani, M (6 July 2017).
307:
1827:
Hao GF, Wang F, Li H, Zhu XL, Yang WC, Huang LS, et al. (2012). "Computational discovery of picomolar Q(o) site inhibitors of cytochrome bc1 complex".
3105:
3701:
2729:
1104:, two protons are consumed from the matrix (M), four protons are released into the inter membrane space (IM) and two electrons are passed to cytochrome
2069:
Skulachev VP (May 1996). "Role of uncoupled and non-coupled oxidations in maintenance of safely low levels of oxygen and its one-electron reductants".
4059:
4011:
3696:
1663:
3455:
2007:
Holmes, J. H.; Sapeika, N; Zwarenstein, H (1975). "Inhibitory effect of anti-obesity drugs on NADH dehydrogenase of mouse heart homogenates".
1991:
1923:
56:
3264:
3213:
1906:, Roberts AG, Muller F, Cape J, Bowman MK (2004). "Q-Cycle Bypass Reactions at the Qo Site of the Cytochrome bc1 (And Related) Complexes".
1776:
Zhang Z, Huang L, Shulmeister VM, Chi YI, Kim KK, Hung LW, et al. (1998). "Electron transfer by domain movement in cytochrome bc1".
4473:
3136:
3555:
3490:
2869:
2788:
4483:
464:, specifically those acting on diphenols and related substances as donor with a cytochrome as acceptor. This enzyme participates in
4167:
3500:
3325:
1522:
3826:
3624:
3259:
2522:
Marres CM, Slater EC (1977). "Polypeptide composition of purified QH2:cytochrome c oxidoreductase from beef-heart mitochondria".
1612:
Mutations in complex III-related genes typically manifest as exercise intolerance. Other mutations have been reported to cause
300:
4488:
3993:
3821:
3485:
3206:
2676:
2034:
Muller, F. L.; Lustgarten, M. S.; Jang, Y.; Richardson, A. & Van Remmen, H. (2007). "Trends in oxidative aging theories".
3816:
3602:
3560:
3026:
2856:
2844:
2793:
2649:
1903:
1649:
1145:. In the complete mechanism, two electrons are transferred from ubiquinol to ubiquinone, via two cytochrome c intermediates.
227:
4323:
251:
4478:
2865:
2684:
1238:
Cytochrome c diffuses. The first ubiquinol (now oxidised to ubiquinone) is released, whilst the semiquinone remains bound.
4438:
3406:
3348:
3238:
2852:
1406:
2210:"Mitochondrial respiratory chain-dependent generation of superoxide anion and its release into the intermembrane space"
1495:
3460:
2659:
3097:
1334:
to the Rieske Iron sulfur protein. Myxothiazol and stigmatellin bind to distinct but overlapping pockets within the Q
1348:
Stigmatellin binds farther from heme bL and nearer the Rieske Iron sulfur protein, with which it strongly interacts.
3634:
3629:
3433:
3299:
4308:
4424:
4411:
4398:
4385:
4372:
4359:
4346:
4110:
4087:
4069:
4051:
3359:
3182:
2953:
465:
359:
4318:
4272:
4215:
4042:
3550:
3428:
2948:
2908:
2783:
2705:
784:
434:
352:
348:
245:
138:
2701:
2259:"TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III"
1864:"Purification of a three-subunit ubiquinol-cytochrome c oxidoreductase complex from Paracoccus denitrificans"
4220:
3597:
3384:
2973:
2889:
2800:
1402:
592:
469:
232:
2117:"The nature and mechanism of superoxide production by the electron transport chain: Its relevance to aging"
4118:
3592:
3249:
3177:
2932:
2881:
2848:
2622:
1031:) is retained in the complex as subunit 9. Thus subunits 10 and 11 correspond to fungal QCR9p and QCR10p.
412:
4241:
4160:
3966:
3706:
3582:
3418:
3396:
2826:
1613:
312:
4313:
1621:
1256:
heme each pull an electron off the bound ubiquinol, releasing two protons into the intermembrane space.
1205:
heme each pull an electron off the bound ubiquinol, releasing two protons into the intermembrane space.
220:
1567:: Ubiquinone binding protein, mutation linked with mitochondrial complex III deficiency nuclear type 3
1289:
The second ubiquinol (now oxidised to ubiquinone), along with the newly formed ubiquinol are released.
3807:
3713:
3570:
3519:
3465:
3161:
2997:
2927:
2815:
1785:
1742:
1644:
375:
155:
69:
1631:
The extent to which these various pathologies are due to bioenergetic deficits or overproduction of
1490:
4277:
4134:
3077:
1235:
Heme transfers its electron to a nearby ubiquinone, resulting in the formation of a ubisemiquinone.
647:
446:
379:
150:
112:
248:
4210:
3737:
3505:
3379:
3151:
2968:
2459:
2416:
2373:
2330:
2094:
1809:
172:
4468:
3727:
3530:
3311:
2756:
2603:
2574:
2539:
2500:
2451:
2408:
2365:
2322:
2280:
2239:
2190:
2146:
2086:
2051:
2016:
1987:
1964:
1929:
1919:
1885:
1844:
1801:
1758:
1715:
1687:
1405:
are highly toxic and are thought to play a role in several pathologies, as well as aging (the
665:
655:
239:
4003:
1960:
4256:
4251:
4225:
4153:
4095:
3540:
3333:
3051:
3046:
2919:
2773:
2595:
2564:
2531:
2490:
2443:
2400:
2357:
2314:
2270:
2229:
2221:
2180:
2136:
2128:
2078:
2043:
1956:
1911:
1875:
1836:
1793:
1750:
1707:
1625:
596:
1282:, whilst the nearby semiquinone produced from round 1 picks up a second electron from the B
4303:
4287:
4200:
3445:
3338:
3128:
2680:
2653:
2633:
1588:
1374:
1126:
500:
383:
362:). Complex III is a multisubunit transmembrane protein encoded by both the mitochondrial (
208:
2721:
2599:
2299:
2047:
1789:
1746:
184:
4452:
4341:
4282:
4034:
2694:
2318:
2234:
2209:
2141:
2116:
1028:
1027:
In vertebrates, a cleavage product of 8 kDa from the N-terminus of the Rieske protein (
583:
488:
461:
283:
143:
1947:
Crofts AR (2004). "The cytochrome bc1 complex: function in the context of structure".
1915:
1880:
1863:
263:
93:
4462:
4246:
4205:
3229:
2670:
2569:
2552:
2535:
638:
473:
258:
61:
2420:
2334:
2098:
1018:
994:
970:
946:
922:
898:
869:
862:
838:
831:
802:
795:
765:
741:
4195:
3862:
3837:
3495:
3374:
3287:
2992:
2978:
2900:
2586:
Wikstrom M, Krab K, Saraste M (1981). "Proton-translocating cytochrome complexes".
2477:
Fisher N, Castleden CK, Bourges I, Brasseur G, Dujardin G, Meunier B (March 2004).
2463:
2377:
2169:"Complex III releases superoxide to both sides of the inner mitochondrial membrane"
1813:
1552:
1451:
1359:
1323:
1279:
1228:
1049:
600:
480:
454:
442:
367:
363:
2639:
1579:: Core 2, mutations linked to mitochondrial complex III deficiency, nuclear type 5
117:
105:
2275:
2258:
1385:
A small fraction of electrons leave the electron transport chain before reaching
81:
4419:
4354:
4190:
4038:
3132:
2225:
1754:
1418:
1414:
1355:
1319:
267:
4447:
646:), and the Rieske Iron Sulfur Protein subunit (ISP) has a two iron, two sulfur
3614:
3276:
2836:
2752:
2553:"Composition, structure, and function of complex III of the respiratory chain"
2361:
2132:
2082:
1632:
1455:
1447:
1442:
1438:
1398:
1394:
1386:
1367:
1138:
1134:
1056:
484:
387:
366:) and the nuclear genomes (all other subunits). Complex III is present in the
34:
1441:. The effect of high membrane potential is thought to have a similar effect.
4393:
4367:
3545:
2778:
1302:
1142:
1130:
28:
2504:
2495:
2478:
2455:
2412:
2369:
2326:
2284:
2243:
2194:
2185:
2168:
2150:
2055:
1968:
1933:
1848:
1719:
2607:
2090:
1889:
1805:
1762:
370:
of all animals and all aerobic eukaryotes and the inner membranes of most
65:
3691:
3565:
3198:
3187:
2810:
2578:
2543:
2020:
1620:, a gene responsible for proper maturation of complex III, can result in
1342:
371:
1040:
787:
355:
4100:
3851:
3846:
3766:
1558:
1101:
1060:
959:
778:
706:
450:
395:
391:
215:
196:
2404:
1840:
1711:
1691:
1116:
669:
659:
4406:
4176:
3956:
3951:
3921:
3916:
3796:
3776:
3771:
3761:
3756:
3751:
3746:
2759:
2447:
1594:
1582:
1576:
1536:
1434:
1429:
site, which, in turn, causes the steady-state concentrations of the Q
1390:
1309:
site and inhibits the transfer of electrons in
Complex III from heme
1007:
983:
851:
820:
730:
295:
191:
179:
167:
2712:#124000 MITOCHONDRIAL COMPLEX III DEFICIENCY, NUCLEAR TYPE 1; MC3DN1
2711:
1543:
encoded cytochrome b; mutations associated with exercise intolerance
1425:
hemes in the reduced state by preventing their re-oxidation at the Q
33:
Crystal structure of mitochondrial cytochrome bc complex bound with
4380:
3976:
3971:
3961:
3946:
3941:
3936:
3931:
3926:
3911:
3906:
3901:
3896:
3891:
3886:
3881:
3876:
3871:
3667:
3654:
3061:
3056:
2479:"Human disease-related mutations in cytochrome b studied in yeast"
1797:
1617:
1600:
1570:
1564:
1540:
1115:
1039:
935:
911:
887:
582:
401:
Ubiquinol—cytochrome-c reductase catalyzes the chemical reaction
3041:
3036:
3031:
3021:
3011:
2804:
2715:
2698:- Calculated positions of bc1 and related complexes in membranes
1546:
1263:
from the 2Fe/2S centre, whilst another is transferred from the B
1212:
from the 2Fe/2S centre, whilst another is transferred from the B
1015:
991:
967:
943:
919:
895:
866:
859:
835:
828:
799:
792:
762:
754:
738:
711:
612:
203:
76:
4149:
4007:
3202:
3101:
2725:
2009:
1473:
1397:. The relevance of this otherwise minor side reaction is that
1363:
1129:
is formed across the membrane. In the overall reaction, two
358:), playing a critical role in biochemical generation of ATP (
1341:
Myxothiazol binds nearer to cytochrome bL (hence termed a "
1330:
site and inhibits the transfer of electrons from reduced QH
1298:
There are three distinct groups of
Complex III inhibitors.
591:
Compared to the other major proton-pumping subunits of the
4145:
1063:
from the mitochondrial matrix to the intermembrane space:
1910:. Methods in Enzymology. Vol. 382. pp. 21–45.
1188:
The reaction proceeds according to the following steps:
416:
1986:(3rd ed.). San Diego: Academic. pp. 114–117.
521:
reduced ubiquinone-cytochrome c reductase, complex III,
1433:
semiquinone to rise, the latter species reacting with
783:
Cytochrome b-c1 complex subunit Rieske, mitochondrial
4436:
2348:
DiMauro S (June 2007). "Mitochondrial DNA medicine".
2162:
2160:
415:
4332:
4296:
4265:
4234:
4183:
4127:
4109:
4086:
4068:
4050:
3860:
3835:
3805:
3785:
3735:
3726:
3680:
3643:
3611:
3579:
3527:
3518:
3474:
3442:
3415:
3393:
3356:
3347:
3324:
3296:
3273:
3246:
3237:
3170:
3144:
3070:
2961:
2945:
2917:
2898:
2879:
2834:
2825:
2766:
306:
294:
282:
277:
257:
238:
226:
214:
202:
190:
178:
166:
161:
149:
137:
132:
127:
111:
99:
87:
75:
55:
47:
42:
21:
2718:; lists all other types of complex III deficiency
2110:
2108:
1231:(not to be confused with cytochrome c1), and the B
421:
1616:and multisystem disorders. However, mutations in
1354:Some have been commercialized as fungicides (the
3083:Electron-transferring-flavoprotein dehydrogenase
2167:Muller FL, Liu Y, Van Remmen H (November 2004).
1198:Cytochrome b binds a ubiquinol and a ubiquinone.
892:Cytochrome b-c1 complex subunit 6, mitochondrial
856:Cytochrome b-c1 complex subunit 2, mitochondrial
825:Cytochrome b-c1 complex subunit 1, mitochondrial
2988:Complex III/Coenzyme Q - cytochrome c reductase
1608:Mutations in complex III genes in human disease
1044:schematic illustration of complex III reactions
539:reduced ubiquinone-cytochrome c oxidoreductase,
2673: (archived August 27, 1999) at scripps.edu
4161:
4019:
3214:
3113:
2737:
2208:Han D, Williams E, Cadenas E (January 2001).
1286:heme, along with two protons from the matrix.
546:mitochondrial electron transport complex III,
8:
1982:Ferguson SJ, Nicholls D, Ferguson S (2002).
1249:A second ubiquinol is bound by cytochrome b.
2683: (archived January 12, 2009) (Requires
1259:One electron is transferred to cytochrome c
1208:One electron is transferred to cytochrome c
689:Table of subunit composition of complex III
587:Structure of complex III (click to enlarge)
564:ubihydroquinol:cytochrome c oxidoreductase,
4168:
4154:
4146:
4026:
4012:
4004:
3732:
3702:Mitochondrial permeability transition pore
3684:
3524:
3353:
3243:
3221:
3207:
3199:
3120:
3106:
3098:
2958:
2831:
2744:
2730:
2722:
2691:UMich Orientation of Proteins in Membranes
2677:Interactive Molecular Model of Complex III
2636: (archived October 9, 2006) at lbl.gov
1409:). Electron leakage occurs mainly at the Q
759:Cytochrome c1, heme protein, mitochondrial
552:ubiquinone-cytochrome b-c1 oxidoreductase,
533:reduced coenzyme Q-cytochrome c reductase,
505:ubiquinol:ferricytochrome-c oxidoreductase
378:as well as multisystem disorders. The bc1
274:
27:
4060:Trans-acenaphthene-1,2-diol dehydrogenase
2704:at the U.S. National Library of Medicine
2568:
2494:
2274:
2233:
2184:
2140:
1879:
1523:Learn how and when to remove this message
964:(N-terminal of Rieske, no separate entry)
518:dihydrocoenzyme Q-cytochrome c reductase,
414:
3697:Mitochondrial membrane transport protein
1961:10.1146/annurev.physiol.66.032102.150251
1366:inhibitors) and as anti-malaria agents (
692:
567:coenzyme QH2-cytochrome c reductase, and
549:ubiquinol-cytochrome c-2 oxidoreductase,
4443:
1679:
1659:
1181:4 x H released into intermembrane space
1059:(CoQ) and the concomitant pumping of 4
558:ubiquinol-cytochrome c1 oxidoreductase,
536:ubiquinone-cytochrome c oxidoreductase,
3456:Cholesterol side-chain cleavage enzyme
3135:: Oxidoreduction-driven transporters (
876:Low-molecular weight protein subunits
530:ubiquinol-cytochrome c oxidoreductase,
507:. Other names in common use include:
124:
18:
460:This enzyme belongs to the family of
7:
1908:Quinones and Quinone Enzymes, Part B
1454:rather than as membrane impermeable
1358:derivatives, best known of which is
382:contains 11 subunits, 3 respiratory
4078:Coenzyme Q - cytochrome c reductase
3370:Coenzyme Q – cytochrome c reductase
2600:10.1146/annurev.bi.50.070181.003203
2048:10.1016/j.freeradbiomed.2007.03.034
1500:Should get merged into table above.
524:(mitochondrial electron transport),
453:(Q), ferro- (Fe) cytochrome c, and
422:{\displaystyle \rightleftharpoons }
3556:Oxoglutarate dehydrogenase complex
3491:Glycerol-3-phosphate dehydrogenase
2974:Complex II/Succinate dehydrogenase
2870:Pyruvate dehydrogenase phosphatase
2319:10.1097/01.bor.0000245729.17759.f2
1316:to oxidized Q (Qi site inhibitor).
1012:Cytochrome b-c1 complex subunit 10
561:CoQH2-cytochrome c oxidoreductase,
555:ubiquinol-cytochrome c2 reductase,
527:ubiquinone-cytochrome c reductase,
515:coenzyme Q-cytochrome c reductase,
481:cytochrome b-562, cytochrome b-566
14:
3501:Carnitine palmitoyltransferase II
2702:Coenzyme+Q-Cytochrome-c+Reductase
1377:inhibits cytochrome c reductase.
988:Cytochrome b-c1 complex subunit 9
940:Cytochrome b-c1 complex subunit 8
916:Cytochrome b-c1 complex subunit 7
374:. Mutations in Complex III cause
4446:
3625:Carbamoyl phosphate synthetase I
3265:Long-chain-fatty-acid—CoA ligase
3260:Carnitine palmitoyltransferase I
1662:
1478:
1389:. Premature electron leakage to
570:QH2:cytochrome c oxidoreductase.
128:ubiquinol—cytochrome-c reductase
3486:Glutamate aspartate transporter
2998:Complex IV/Cytochrome c oxidase
2647:complex site (Antony R. Crofts)
1278:then transfers its electron to
3603:Pyruvate dehydrogenase complex
3561:Succinyl coenzyme A synthetase
2845:Pyruvate dehydrogenase complex
2630:complex site (Edward A. Berry)
1862:Yang XH, Trumpower BL (1986).
1650:Photosynthetic reaction centre
1048:It catalyzes the reduction of
347:, is the third complex in the
1:
2866:Pyruvate dehydrogenase kinase
2660:PROMISE Database: cytochrome
1916:10.1016/S0076-6879(04)82002-0
1881:10.1016/S0021-9258(18)67236-9
719:Respiratory subunit proteins
437:of this enzyme are quinol (QH
429:Q + 2 ferrocytochrome c + 2 H
326:coenzyme Q : cytochrome
3407:Dihydroorotate dehydrogenase
2969:Complex I/NADH dehydrogenase
2570:10.1016/0304-4173(76)90012-4
2536:10.1016/0005-2728(77)90099-8
2276:10.1016/j.molcel.2017.06.001
1561:: Rieske iron sulfur protein
1407:free radical theory of aging
1393:results in the formation of
390:C1, Rieske protein), 2 core
3461:Steroid 11-beta-hydroxylase
2298:DiMauro S (November 2006).
1755:10.1126/science.281.5373.64
1498:. The specific problem is:
1184:2 x H picked up from matrix
653:Structures of complex III:
394:and 6 low-molecular weight
4505:
4474:Enzymes of known structure
3635:N-Acetylglutamate synthase
3630:Ornithine transcarbamylase
3434:Glycerol phosphate shuttle
3300:monoamine neurotransmitter
2789:Oxoglutarate dehydrogenase
2300:"Mitochondrial myopathies"
1494:to meet Knowledge (XXG)'s
1413:site and is stimulated by
1227:transfers its electron to
714:family with Human protein
4324:Michaelis–Menten kinetics
3989:
3687:
3663:
3360:oxidative phosphorylation
3183:Inorganic pyrophosphatase
2954:oxidative phosphorylation
2362:10.1007/s10540-007-9032-5
2226:10.1042/0264-6021:3530411
2133:10.1007/s11357-000-0022-9
2083:10.1017/s0033583500005795
1294:Inhibitors of complex III
875:
808:
718:
705:Protein description from
466:oxidative phosphorylation
360:oxidative phosphorylation
273:
26:
4216:Diffusion-limited enzyme
3551:Isocitrate dehydrogenase
3429:Malate-aspartate shuttle
2949:electron transport chain
2909:Methylmalonyl-CoA mutase
2784:Isocitrate dehydrogenase
2706:Medical Subject Headings
593:electron transport chain
503:of this enzyme class is
349:electron transport chain
3598:Glutamate dehydrogenase
3385:Succinate dehydrogenase
2890:Glutamate dehydrogenase
2801:Succinate dehydrogenase
2794:Succinyl CoA synthetase
1403:reactive oxygen species
1252:The 2Fe/2S center and B
1201:The 2Fe/2S center and B
332:, sometimes called the
22:Cytochrome b-c1 complex
4489:Transmembrane proteins
4119:Cytochrome b6f complex
3994:mitochondrial diseases
3593:Aspartate transaminase
3250:fatty acid degradation
3178:Cytochrome b6f complex
2933:Aspartate transaminase
2695:families/superfamily-3
2557:Biochim. Biophys. Acta
2524:Biochim. Biophys. Acta
2496:10.1074/jbc.M313866200
2186:10.1074/jbc.M407715200
1635:is presently unknown.
1450:as membrane permeable
1121:
1100:In the process called
1045:
809:Core protein subunits
677:Composition of complex
588:
423:
409:+ 2 ferricytochrome c
4309:Eadie–Hofstee diagram
4242:Allosteric regulation
3707:Mitochondrial carrier
3583:anaplerotic reactions
3419:mitochondrial shuttle
3397:pyrimidine metabolism
2036:Free Radic. Biol. Med
1614:septo-optic dysplasia
1119:
1043:
681:In vertebrates the bc
586:
424:
343:, and at other times
4484:Iron–sulfur proteins
4479:Cellular respiration
4319:Lineweaver–Burk plot
3714:Translocator protein
3571:Malate dehydrogenase
3466:Aldosterone synthase
2928:Pyruvate carboxylase
2816:Malate dehydrogenase
1645:Cellular respiration
1505:improve this section
1381:Oxygen free radicals
413:
376:exercise intolerance
4135:Alternative oxidase
3326:Intermembrane space
3078:Alternative oxidase
2882:α-ketoglutaric acid
2307:Curr Opin Rheumatol
1790:1998Natur.392..677Z
1747:1998Sci...281...64I
1084:→ Q + 2 cytochrome
648:iron-sulfur cluster
4278:Enzyme superfamily
4211:Enzyme promiscuity
3681:Other/to be sorted
3646:alcohol metabolism
3506:Uncoupling protein
3380:NADH dehydrogenase
2652:2007-09-17 at the
2588:Annu. Rev. Biochem
2551:Rieske JS (1976).
1949:Annu. Rev. Physiol
1622:Björnstad syndrome
1122:
1112:Reaction mechanism
1046:
702:Human gene symbol
629:), the cytochrome
589:
441:) and ferri- (Fe)
419:
4434:
4433:
4143:
4142:
4001:
4000:
3985:
3984:
3728:Mitochondrial DNA
3722:
3721:
3676:
3675:
3531:citric acid cycle
3514:
3513:
3320:
3319:
3312:Monoamine oxidase
3196:
3195:
3095:
3094:
3091:
3090:
2941:
2940:
2757:Citric acid cycle
2405:10.1002/ana.10151
2115:Muller F (2000).
1993:978-0-12-518121-1
1925:978-0-12-182786-1
1841:10.1021/ja3001908
1712:10.1021/bi0341814
1656:Additional images
1533:
1532:
1525:
1496:quality standards
1487:This section may
1024:
1023:
597:prosthetic groups
576:
575:
322:
321:
318:
317:
221:metabolic pathway
123:
122:
4496:
4451:
4450:
4442:
4314:Hanes–Woolf plot
4257:Enzyme activator
4252:Enzyme inhibitor
4226:Enzyme catalysis
4170:
4163:
4156:
4147:
4096:Catechol oxidase
4028:
4021:
4014:
4005:
3865:
3840:
3810:
3790:
3740:
3733:
3685:
3648:
3618:
3586:
3541:Citrate synthase
3534:
3525:
3479:
3449:
3422:
3400:
3363:
3354:
3334:Adenylate kinase
3305:
3281:
3253:
3244:
3223:
3216:
3209:
3200:
3122:
3115:
3108:
3099:
2959:
2920:oxaloacetic acid
2832:
2774:Citrate synthase
2746:
2739:
2732:
2723:
2611:
2582:
2572:
2547:
2509:
2508:
2498:
2474:
2468:
2467:
2448:10.1002/ana.1224
2431:
2425:
2424:
2388:
2382:
2381:
2345:
2339:
2338:
2304:
2295:
2289:
2288:
2278:
2269:(1): 96–105.e4.
2254:
2248:
2247:
2237:
2205:
2199:
2198:
2188:
2179:(47): 49064–73.
2164:
2155:
2154:
2144:
2112:
2103:
2102:
2066:
2060:
2059:
2031:
2025:
2024:
2004:
1998:
1997:
1979:
1973:
1972:
1944:
1938:
1937:
1900:
1894:
1893:
1883:
1859:
1853:
1852:
1835:(27): 11168–76.
1824:
1818:
1817:
1784:(6677): 677–84.
1773:
1767:
1766:
1730:
1724:
1723:
1694:
1684:
1666:
1626:GRACILE syndrome
1528:
1521:
1517:
1514:
1508:
1482:
1481:
1474:
1470:Human gene names
1464:
1463:
1133:are oxidized to
1096:
1095:
1083:
1082:
1055:by oxidation of
693:
672:
662:
633:subunit has one
510:
509:
445:, whereas its 3
428:
426:
425:
420:
330:– oxidoreductase
275:
125:
31:
19:
16:Class of enzymes
4504:
4503:
4499:
4498:
4497:
4495:
4494:
4493:
4459:
4458:
4457:
4445:
4437:
4435:
4430:
4342:Oxidoreductases
4328:
4304:Enzyme kinetics
4292:
4288:List of enzymes
4261:
4230:
4201:Catalytic triad
4179:
4174:
4144:
4139:
4123:
4105:
4082:
4064:
4046:
4035:Oxidoreductases
4032:
4002:
3997:
3981:
3861:
3856:
3836:
3831:
3806:
3801:
3786:
3781:
3736:
3718:
3672:
3659:
3644:
3639:
3612:
3607:
3580:
3575:
3528:
3510:
3475:
3470:
3446:steroidogenesis
3443:
3438:
3416:
3411:
3394:
3389:
3357:
3343:
3339:Creatine kinase
3316:
3302:
3297:
3292:
3274:
3269:
3247:
3233:
3227:
3197:
3192:
3166:
3157:ETC Complex III
3140:
3126:
3096:
3087:
3066:
3008:
2982:
2952:
2947:
2937:
2913:
2894:
2875:
2821:
2762:
2750:
2681:Wayback Machine
2666:
2654:Wayback Machine
2646:
2634:Wayback Machine
2629:
2619:
2614:
2585:
2550:
2521:
2517:
2515:Further reading
2512:
2489:(13): 12951–8.
2476:
2475:
2471:
2433:
2432:
2428:
2390:
2389:
2385:
2347:
2346:
2342:
2302:
2297:
2296:
2292:
2256:
2255:
2251:
2220:(Pt 2): 411–6.
2207:
2206:
2202:
2166:
2165:
2158:
2114:
2113:
2106:
2071:Q. Rev. Biophys
2068:
2067:
2063:
2033:
2032:
2028:
2006:
2005:
2001:
1994:
1981:
1980:
1976:
1946:
1945:
1941:
1926:
1902:
1901:
1897:
1874:(26): 12282–9.
1861:
1860:
1856:
1826:
1825:
1821:
1775:
1774:
1770:
1741:(5373): 64–71.
1732:
1731:
1727:
1706:(30): 9067–80.
1696:
1686:
1685:
1681:
1677:
1670:
1667:
1658:
1641:
1610:
1597:: 7.2KD subunit
1591:: 6.4KD subunit
1573:: hinge protein
1549:: cytochrome c1
1529:
1518:
1512:
1509:
1502:
1483:
1479:
1472:
1462:
1459:
1458:
1457:
1432:
1428:
1412:
1383:
1375:propylhexedrine
1337:
1333:
1329:
1315:
1308:
1296:
1285:
1277:
1270:
1266:
1262:
1255:
1234:
1226:
1219:
1215:
1211:
1204:
1172:
1158:
1127:proton gradient
1114:
1094:
1091:
1090:
1089:
1081:
1078:
1077:
1076:
1071:+ 2 cytochrome
1070:
1038:
691:
684:
679:
664:
654:
644:
628:
621:
581:
501:systematic name
497:
483:, and a 2-Iron
477:
468:. It has four
462:oxidoreductases
440:
411:
410:
408:
386:(cytochrome B,
340:
89:OPM superfamily
38:
17:
12:
11:
5:
4502:
4500:
4492:
4491:
4486:
4481:
4476:
4471:
4461:
4460:
4456:
4455:
4432:
4431:
4429:
4428:
4415:
4402:
4389:
4376:
4363:
4350:
4336:
4334:
4330:
4329:
4327:
4326:
4321:
4316:
4311:
4306:
4300:
4298:
4294:
4293:
4291:
4290:
4285:
4280:
4275:
4269:
4267:
4266:Classification
4263:
4262:
4260:
4259:
4254:
4249:
4244:
4238:
4236:
4232:
4231:
4229:
4228:
4223:
4218:
4213:
4208:
4203:
4198:
4193:
4187:
4185:
4181:
4180:
4175:
4173:
4172:
4165:
4158:
4150:
4141:
4140:
4138:
4137:
4131:
4129:
4125:
4124:
4122:
4121:
4115:
4113:
4107:
4106:
4104:
4103:
4098:
4092:
4090:
4084:
4083:
4081:
4080:
4074:
4072:
4066:
4065:
4063:
4062:
4056:
4054:
4048:
4047:
4033:
4031:
4030:
4023:
4016:
4008:
3999:
3998:
3990:
3987:
3986:
3983:
3982:
3980:
3979:
3974:
3969:
3964:
3959:
3954:
3949:
3944:
3939:
3934:
3929:
3924:
3919:
3914:
3909:
3904:
3899:
3894:
3889:
3884:
3879:
3874:
3868:
3866:
3858:
3857:
3855:
3854:
3849:
3843:
3841:
3833:
3832:
3830:
3829:
3824:
3819:
3813:
3811:
3803:
3802:
3800:
3799:
3793:
3791:
3783:
3782:
3780:
3779:
3774:
3769:
3764:
3759:
3754:
3749:
3743:
3741:
3730:
3724:
3723:
3720:
3719:
3717:
3716:
3711:
3710:
3709:
3704:
3694:
3688:
3682:
3678:
3677:
3674:
3673:
3671:
3670:
3664:
3661:
3660:
3658:
3657:
3651:
3649:
3641:
3640:
3638:
3637:
3632:
3627:
3621:
3619:
3609:
3608:
3606:
3605:
3600:
3595:
3589:
3587:
3577:
3576:
3574:
3573:
3568:
3563:
3558:
3553:
3548:
3543:
3537:
3535:
3522:
3516:
3515:
3512:
3511:
3509:
3508:
3503:
3498:
3493:
3488:
3482:
3480:
3472:
3471:
3469:
3468:
3463:
3458:
3452:
3450:
3440:
3439:
3437:
3436:
3431:
3425:
3423:
3413:
3412:
3410:
3409:
3403:
3401:
3391:
3390:
3388:
3387:
3382:
3377:
3372:
3366:
3364:
3351:
3349:Inner membrane
3345:
3344:
3342:
3341:
3336:
3330:
3328:
3322:
3321:
3318:
3317:
3315:
3314:
3308:
3306:
3294:
3293:
3291:
3290:
3284:
3282:
3271:
3270:
3268:
3267:
3262:
3256:
3254:
3241:
3239:Outer membrane
3235:
3234:
3228:
3226:
3225:
3218:
3211:
3203:
3194:
3193:
3191:
3190:
3185:
3180:
3174:
3172:
3168:
3167:
3165:
3164:
3162:ETC Complex IV
3159:
3154:
3148:
3146:
3142:
3141:
3127:
3125:
3124:
3117:
3110:
3102:
3093:
3092:
3089:
3088:
3086:
3085:
3080:
3074:
3072:
3068:
3067:
3065:
3064:
3059:
3054:
3049:
3044:
3039:
3034:
3029:
3024:
3019:
3014:
3006:
3001:
3000:
2995:
2990:
2985:
2980:
2976:
2971:
2965:
2963:
2956:
2943:
2942:
2939:
2938:
2936:
2935:
2930:
2924:
2922:
2915:
2914:
2912:
2911:
2905:
2903:
2896:
2895:
2893:
2892:
2886:
2884:
2877:
2876:
2874:
2873:
2864:(regulated by
2861:
2860:
2841:
2839:
2829:
2823:
2822:
2820:
2819:
2813:
2808:
2797:
2796:
2791:
2786:
2781:
2776:
2770:
2768:
2764:
2763:
2751:
2749:
2748:
2741:
2734:
2726:
2720:
2719:
2709:
2699:
2688:
2674:
2664:
2657:
2644:
2637:
2627:
2618:
2617:External links
2615:
2613:
2612:
2583:
2563:(2): 195–247.
2548:
2530:(3): 531–548.
2518:
2516:
2513:
2511:
2510:
2469:
2426:
2383:
2340:
2290:
2263:Molecular Cell
2249:
2200:
2156:
2127:(4): 227–253.
2104:
2077:(2): 169–202.
2061:
2042:(4): 477–503.
2026:
1999:
1992:
1974:
1939:
1924:
1895:
1854:
1819:
1768:
1725:
1678:
1676:
1673:
1672:
1671:
1668:
1661:
1657:
1654:
1653:
1652:
1647:
1640:
1637:
1609:
1606:
1605:
1604:
1598:
1592:
1586:
1580:
1574:
1568:
1562:
1556:
1555:: cytochrome c
1550:
1544:
1531:
1530:
1486:
1484:
1477:
1471:
1468:
1460:
1430:
1426:
1410:
1382:
1379:
1352:
1351:
1350:
1349:
1346:
1335:
1331:
1327:
1326:binds to the Q
1317:
1313:
1306:
1305:binds to the Q
1295:
1292:
1291:
1290:
1287:
1283:
1275:
1272:
1268:
1264:
1260:
1257:
1253:
1250:
1240:
1239:
1236:
1232:
1224:
1221:
1217:
1213:
1209:
1206:
1202:
1199:
1186:
1185:
1182:
1179:
1173:
1170:
1163:
1156:
1141:is reduced to
1113:
1110:
1098:
1097:
1092:
1079:
1068:
1037:
1034:
1033:
1032:
1029:Signal peptide
1022:
1021:
1013:
1010:
1005:
1002:
998:
997:
989:
986:
981:
978:
974:
973:
965:
962:
957:
954:
950:
949:
941:
938:
933:
930:
926:
925:
917:
914:
909:
906:
902:
901:
893:
890:
885:
882:
878:
877:
873:
872:
857:
854:
849:
846:
842:
841:
826:
823:
818:
815:
811:
810:
806:
805:
790:
781:
776:
773:
769:
768:
760:
757:
752:
749:
745:
744:
736:
733:
728:
727:MT-CYB / Cyt b
725:
721:
720:
716:
715:
709:
703:
700:
697:
690:
687:
682:
678:
675:
642:
626:
619:
580:
577:
574:
573:
572:
571:
568:
565:
562:
559:
556:
553:
550:
547:
542:
541:
540:
537:
534:
531:
528:
525:
522:
519:
516:
496:
493:
475:
438:
433:Thus, the two
431:
430:
418:
406:
338:
320:
319:
316:
315:
310:
304:
303:
298:
292:
291:
286:
280:
279:
271:
270:
261:
255:
254:
243:
236:
235:
230:
224:
223:
218:
212:
211:
206:
200:
199:
194:
188:
187:
182:
176:
175:
170:
164:
163:
159:
158:
153:
147:
146:
141:
135:
134:
130:
129:
121:
120:
115:
109:
108:
103:
97:
96:
91:
85:
84:
79:
73:
72:
59:
53:
52:
49:
45:
44:
40:
39:
32:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
4501:
4490:
4487:
4485:
4482:
4480:
4477:
4475:
4472:
4470:
4467:
4466:
4464:
4454:
4449:
4444:
4440:
4426:
4422:
4421:
4416:
4413:
4409:
4408:
4403:
4400:
4396:
4395:
4390:
4387:
4383:
4382:
4377:
4374:
4370:
4369:
4364:
4361:
4357:
4356:
4351:
4348:
4344:
4343:
4338:
4337:
4335:
4331:
4325:
4322:
4320:
4317:
4315:
4312:
4310:
4307:
4305:
4302:
4301:
4299:
4295:
4289:
4286:
4284:
4283:Enzyme family
4281:
4279:
4276:
4274:
4271:
4270:
4268:
4264:
4258:
4255:
4253:
4250:
4248:
4247:Cooperativity
4245:
4243:
4240:
4239:
4237:
4233:
4227:
4224:
4222:
4219:
4217:
4214:
4212:
4209:
4207:
4206:Oxyanion hole
4204:
4202:
4199:
4197:
4194:
4192:
4189:
4188:
4186:
4182:
4178:
4171:
4166:
4164:
4159:
4157:
4152:
4151:
4148:
4136:
4133:
4132:
4130:
4126:
4120:
4117:
4116:
4114:
4112:
4108:
4102:
4099:
4097:
4094:
4093:
4091:
4089:
4085:
4079:
4076:
4075:
4073:
4071:
4067:
4061:
4058:
4057:
4055:
4053:
4049:
4044:
4040:
4036:
4029:
4024:
4022:
4017:
4015:
4010:
4009:
4006:
3996:
3995:
3988:
3978:
3975:
3973:
3970:
3968:
3965:
3963:
3960:
3958:
3955:
3953:
3950:
3948:
3945:
3943:
3940:
3938:
3935:
3933:
3930:
3928:
3925:
3923:
3920:
3918:
3915:
3913:
3910:
3908:
3905:
3903:
3900:
3898:
3895:
3893:
3890:
3888:
3885:
3883:
3880:
3878:
3875:
3873:
3870:
3869:
3867:
3864:
3859:
3853:
3850:
3848:
3845:
3844:
3842:
3839:
3834:
3828:
3825:
3823:
3820:
3818:
3815:
3814:
3812:
3809:
3804:
3798:
3795:
3794:
3792:
3789:
3784:
3778:
3775:
3773:
3770:
3768:
3765:
3763:
3760:
3758:
3755:
3753:
3750:
3748:
3745:
3744:
3742:
3739:
3734:
3731:
3729:
3725:
3715:
3712:
3708:
3705:
3703:
3700:
3699:
3698:
3695:
3693:
3690:
3689:
3686:
3683:
3679:
3669:
3666:
3665:
3662:
3656:
3653:
3652:
3650:
3647:
3642:
3636:
3633:
3631:
3628:
3626:
3623:
3622:
3620:
3617:
3616:
3610:
3604:
3601:
3599:
3596:
3594:
3591:
3590:
3588:
3585:
3584:
3578:
3572:
3569:
3567:
3564:
3562:
3559:
3557:
3554:
3552:
3549:
3547:
3544:
3542:
3539:
3538:
3536:
3533:
3532:
3526:
3523:
3521:
3517:
3507:
3504:
3502:
3499:
3497:
3494:
3492:
3489:
3487:
3484:
3483:
3481:
3478:
3473:
3467:
3464:
3462:
3459:
3457:
3454:
3453:
3451:
3448:
3447:
3441:
3435:
3432:
3430:
3427:
3426:
3424:
3421:
3420:
3414:
3408:
3405:
3404:
3402:
3399:
3398:
3392:
3386:
3383:
3381:
3378:
3376:
3373:
3371:
3368:
3367:
3365:
3362:
3361:
3355:
3352:
3350:
3346:
3340:
3337:
3335:
3332:
3331:
3329:
3327:
3323:
3313:
3310:
3309:
3307:
3304:
3301:
3295:
3289:
3286:
3285:
3283:
3280:
3278:
3272:
3266:
3263:
3261:
3258:
3257:
3255:
3252:
3251:
3245:
3242:
3240:
3236:
3231:
3230:Mitochondrial
3224:
3219:
3217:
3212:
3210:
3205:
3204:
3201:
3189:
3186:
3184:
3181:
3179:
3176:
3175:
3173:
3169:
3163:
3160:
3158:
3155:
3153:
3152:ETC Complex I
3150:
3149:
3147:
3143:
3138:
3134:
3130:
3123:
3118:
3116:
3111:
3109:
3104:
3103:
3100:
3084:
3081:
3079:
3076:
3075:
3073:
3069:
3063:
3060:
3058:
3055:
3053:
3050:
3048:
3045:
3043:
3040:
3038:
3035:
3033:
3030:
3028:
3025:
3023:
3020:
3018:
3015:
3013:
3009:
3003:
3002:
2999:
2996:
2994:
2991:
2989:
2986:
2984:
2977:
2975:
2972:
2970:
2967:
2966:
2964:
2960:
2957:
2955:
2950:
2946:Mitochondrial
2944:
2934:
2931:
2929:
2926:
2925:
2923:
2921:
2916:
2910:
2907:
2906:
2904:
2902:
2897:
2891:
2888:
2887:
2885:
2883:
2878:
2871:
2867:
2863:
2862:
2858:
2854:
2850:
2846:
2843:
2842:
2840:
2838:
2833:
2830:
2828:
2824:
2817:
2814:
2812:
2809:
2806:
2802:
2799:
2798:
2795:
2792:
2790:
2787:
2785:
2782:
2780:
2777:
2775:
2772:
2771:
2769:
2765:
2761:
2758:
2754:
2747:
2742:
2740:
2735:
2733:
2728:
2727:
2724:
2717:
2713:
2710:
2707:
2703:
2700:
2697:
2696:
2692:
2689:
2686:
2682:
2678:
2675:
2672:
2671:archive.today
2668:
2663:
2658:
2655:
2651:
2648:
2643:
2638:
2635:
2631:
2626:
2621:
2620:
2616:
2609:
2605:
2601:
2597:
2593:
2589:
2584:
2580:
2576:
2571:
2566:
2562:
2558:
2554:
2549:
2545:
2541:
2537:
2533:
2529:
2525:
2520:
2519:
2514:
2506:
2502:
2497:
2492:
2488:
2484:
2483:J. Biol. Chem
2480:
2473:
2470:
2465:
2461:
2457:
2453:
2449:
2445:
2441:
2437:
2430:
2427:
2422:
2418:
2414:
2410:
2406:
2402:
2399:(3): 388–92.
2398:
2394:
2387:
2384:
2379:
2375:
2371:
2367:
2363:
2359:
2355:
2351:
2344:
2341:
2336:
2332:
2328:
2324:
2320:
2316:
2313:(6): 636–41.
2312:
2308:
2301:
2294:
2291:
2286:
2282:
2277:
2272:
2268:
2264:
2260:
2253:
2250:
2245:
2241:
2236:
2231:
2227:
2223:
2219:
2215:
2211:
2204:
2201:
2196:
2192:
2187:
2182:
2178:
2174:
2173:J. Biol. Chem
2170:
2163:
2161:
2157:
2152:
2148:
2143:
2138:
2134:
2130:
2126:
2122:
2118:
2111:
2109:
2105:
2100:
2096:
2092:
2088:
2084:
2080:
2076:
2072:
2065:
2062:
2057:
2053:
2049:
2045:
2041:
2037:
2030:
2027:
2022:
2018:
2014:
2010:
2003:
2000:
1995:
1989:
1985:
1984:Bioenergetics
1978:
1975:
1970:
1966:
1962:
1958:
1954:
1950:
1943:
1940:
1935:
1931:
1927:
1921:
1917:
1913:
1909:
1905:
1899:
1896:
1891:
1887:
1882:
1877:
1873:
1869:
1865:
1858:
1855:
1850:
1846:
1842:
1838:
1834:
1830:
1829:J Am Chem Soc
1823:
1820:
1815:
1811:
1807:
1803:
1799:
1798:10.1038/33612
1795:
1791:
1787:
1783:
1779:
1772:
1769:
1764:
1760:
1756:
1752:
1748:
1744:
1740:
1736:
1729:
1726:
1721:
1717:
1713:
1709:
1705:
1701:
1693:
1689:
1683:
1680:
1674:
1665:
1660:
1655:
1651:
1648:
1646:
1643:
1642:
1638:
1636:
1634:
1629:
1627:
1623:
1619:
1615:
1607:
1602:
1599:
1596:
1593:
1590:
1587:
1584:
1581:
1578:
1575:
1572:
1569:
1566:
1563:
1560:
1557:
1554:
1551:
1548:
1545:
1542:
1538:
1535:
1534:
1527:
1524:
1516:
1513:December 2023
1506:
1501:
1497:
1493:
1492:
1485:
1476:
1475:
1469:
1467:
1465:
1453:
1449:
1444:
1440:
1436:
1424:
1420:
1416:
1408:
1404:
1400:
1396:
1392:
1388:
1380:
1378:
1376:
1371:
1369:
1365:
1361:
1357:
1347:
1345:" inhibitor).
1344:
1340:
1339:
1325:
1321:
1318:
1312:
1304:
1301:
1300:
1299:
1293:
1288:
1281:
1273:
1267:heme to the B
1258:
1251:
1248:
1247:
1246:
1244:
1237:
1230:
1222:
1216:heme to the B
1207:
1200:
1197:
1196:
1195:
1193:
1189:
1183:
1180:
1178:
1174:
1168:
1164:
1161:
1154:
1153:
1152:
1150:
1146:
1144:
1140:
1136:
1132:
1128:
1118:
1111:
1109:
1107:
1103:
1087:
1074:
1066:
1065:
1064:
1062:
1058:
1054:
1053:
1042:
1035:
1030:
1026:
1025:
1020:
1017:
1014:
1011:
1009:
1006:
1003:
1000:
999:
996:
993:
990:
987:
985:
982:
979:
976:
975:
972:
969:
966:
963:
961:
958:
955:
952:
951:
948:
945:
942:
939:
937:
934:
931:
928:
927:
924:
921:
918:
915:
913:
910:
907:
904:
903:
900:
897:
894:
891:
889:
886:
883:
880:
879:
874:
871:
868:
864:
861:
858:
855:
853:
850:
847:
844:
843:
840:
837:
833:
830:
827:
824:
822:
819:
816:
813:
812:
807:
804:
801:
797:
794:
791:
789:
786:
782:
780:
777:
775:Rieske / UCR1
774:
771:
770:
767:
764:
761:
758:
756:
753:
751:CYC1 / Cyt c1
750:
747:
746:
743:
740:
737:
734:
732:
729:
726:
723:
722:
717:
713:
710:
708:
704:
701:
699:Subunit name
698:
695:
694:
688:
686:
676:
674:
671:
667:
661:
657:
651:
649:
645:
641:
636:
632:
625:
618:
614:
610:
606:
604:
598:
594:
585:
578:
569:
566:
563:
560:
557:
554:
551:
548:
545:
544:
543:
538:
535:
532:
529:
526:
523:
520:
517:
514:
513:
512:
511:
508:
506:
502:
494:
492:
490:
486:
482:
478:
471:
467:
463:
458:
456:
452:
448:
444:
436:
404:
403:
402:
399:
397:
393:
389:
385:
381:
377:
373:
369:
365:
361:
357:
354:
350:
346:
342:
337:
331:
329:
314:
311:
309:
305:
302:
299:
297:
293:
290:
287:
285:
281:
276:
272:
269:
265:
262:
260:
259:Gene Ontology
256:
253:
250:
247:
244:
241:
237:
234:
231:
229:
225:
222:
219:
217:
213:
210:
207:
205:
201:
198:
197:NiceZyme view
195:
193:
189:
186:
183:
181:
177:
174:
171:
169:
165:
160:
157:
154:
152:
148:
145:
142:
140:
136:
131:
126:
119:
116:
114:
110:
107:
104:
102:
98:
95:
92:
90:
86:
83:
80:
78:
74:
71:
67:
63:
60:
58:
54:
50:
46:
41:
36:
30:
25:
20:
4420:Translocases
4417:
4404:
4391:
4378:
4365:
4355:Transferases
4352:
4339:
4196:Binding site
4077:
3991:
3838:ATP synthase
3787:
3645:
3613:
3581:
3529:
3496:ATP synthase
3476:
3444:
3417:
3395:
3375:Cytochrome c
3369:
3358:
3298:
3288:Kynureninase
3275:
3248:
3156:
3133:proton pumps
3004:
2993:Cytochrome c
2987:
2901:succinyl-CoA
2693:
2661:
2641:
2624:
2591:
2587:
2560:
2556:
2527:
2523:
2486:
2482:
2472:
2442:(4): 540–3.
2439:
2435:
2429:
2396:
2392:
2386:
2356:(1–3): 5–9.
2353:
2349:
2343:
2310:
2306:
2293:
2266:
2262:
2252:
2217:
2213:
2203:
2176:
2172:
2124:
2120:
2074:
2070:
2064:
2039:
2035:
2029:
2015:(4): 645–6.
2012:
2008:
2002:
1983:
1977:
1952:
1948:
1942:
1907:
1898:
1871:
1867:
1857:
1832:
1828:
1822:
1781:
1777:
1771:
1738:
1734:
1728:
1703:
1700:Biochemistry
1699:
1682:
1630:
1611:
1519:
1510:
1503:Please help
1499:
1488:
1422:
1384:
1372:
1360:azoxystrobin
1353:
1324:stigmatellin
1310:
1297:
1280:cytochrome c
1274:Cytochrome c
1242:
1241:
1229:cytochrome c
1223:Cytochrome c
1191:
1190:
1187:
1176:
1166:
1159:
1148:
1147:
1125:a result, a
1123:
1105:
1099:
1085:
1072:
1051:
1047:
1004:QCR11 / SU11
980:QCR10 / SU10
735:Cytochrome b
680:
652:
639:
637:-type heme (
634:
630:
623:
616:
608:
602:
590:
504:
498:
495:Nomenclature
474:cytochrome c
459:
443:cytochrome c
432:
400:
368:mitochondria
364:cytochrome b
344:
335:
333:
327:
325:
323:
185:BRENDA entry
4191:Active site
3788:Complex III
3010:synthesis:
2827:Anaplerotic
2656:at uiuc.edu
2640:cytochrome
2623:cytochrome
2594:: 623–655.
2436:Ann. Neurol
2393:Ann. Neurol
2350:Biosci. Rep
1955:: 689–733.
1868:J Biol Chem
1507:if you can.
1419:Antimycin A
1415:antimycin A
1356:strobilurin
1320:Myxothiazol
1303:Antimycin A
1135:ubiquinones
1120:The Q cycle
1075:(Fe) + 2 H
1050:cytochrome
601:cytochrome
345:complex III
334:cytochrome
173:IntEnz view
133:Identifiers
101:OPM protein
43:Identifiers
4463:Categories
4394:Isomerases
4368:Hydrolases
4235:Regulation
3808:Complex IV
3615:urea cycle
3303:metabolism
3279:metabolism
3277:tryptophan
3005:Coenzyme Q
2979:Coenzyme Q
2837:acetyl-CoA
2753:Metabolism
2214:Biochem. J
1675:References
1633:superoxide
1448:superoxide
1443:Superoxide
1439:superoxide
1421:locks the
1401:and other
1399:superoxide
1395:superoxide
1387:complex IV
1368:atovaquone
1175:2 x Cyt c
1139:ubiquinone
1131:ubiquinols
1088:(Fe) + 4 H
1057:coenzyme Q
956:QCR9 / SU9
932:QCR8 / SU8
908:QCR7 / SU7
884:QCR6 / SU6
848:QCR2 / SU2
817:QCR1 / SU1
650:(2Fe•2S).
485:ferredoxin
435:substrates
388:cytochrome
242:structures
209:KEGG entry
156:9027-03-6
113:Membranome
35:ubiquinone
4273:EC number
3992:see also
3738:Complex I
3546:Aconitase
2779:Aconitase
2685:MDL Chime
1904:Kramer DM
1695:;
1143:ubiquinol
788:1.10.2.2
663:,
579:Structure
470:cofactors
417:⇌
162:Databases
4469:EC 7.1.1
4297:Kinetics
4221:Cofactor
4184:Activity
4041:family (
4039:diphenol
3692:Frataxin
3566:Fumarase
3232:proteins
3188:V-ATPase
3129:Ion pump
2811:Fumarase
2650:Archived
2505:14718526
2456:11601507
2421:12425236
2413:11891837
2370:17484047
2335:29140366
2327:17053512
2285:28673544
2244:11139407
2195:15317809
2151:23604868
2099:40859585
2056:17640558
1969:14977419
1934:15047094
1849:22690928
1720:12885240
1639:See also
1624:and the
1585:: Core 1
1489:require
1437:to form
1343:proximal
1160:oxidised
1137:and one
1036:Reaction
1019:PF08997
995:PF05365
971:PF09165
947:PF02939
923:PF02271
899:PF02320
803:PF00355
796:PF02921
766:PF02167
742:PF13631
673:
607:has two
447:products
396:proteins
392:proteins
384:subunits
372:bacteria
356:1.10.2.2
313:proteins
301:articles
289:articles
246:RCSB PDB
4453:Biology
4407:Ligases
4177:Enzymes
4111:1.10.99
4101:Laccase
3852:MT-ATP8
3847:MT-ATP6
3767:MT-ND4L
2983:(CoQ10)
2962:Primary
2818:and ETC
2760:enzymes
2679:at the
2667:complex
2632:at the
2608:6267990
2464:8944744
2378:5849380
2235:1221585
2142:3455268
2091:8870073
1890:3017970
1814:4380033
1806:9565029
1786:Bibcode
1763:9651245
1743:Bibcode
1735:Science
1559:UQCRFS1
1491:cleanup
1243:Round 2
1192:Round 1
1177:reduced
1167:reduced
1149:Overall
1102:Q cycle
1061:protons
960:UQCRFS1
870:PF05193
863:PF00675
839:PF05193
832:PF00675
779:UQCRFS1
707:UniProt
605:subunit
487:of the
451:quinone
380:complex
341:complex
268:QuickGO
233:profile
216:MetaCyc
151:CAS no.
144:7.1.1.8
4439:Portal
4381:Lyases
4088:1.10.3
4070:1.10.2
4052:1.10.1
3957:MT-TS2
3952:MT-TS1
3922:MT-TL2
3917:MT-TL1
3827:MT-CO3
3822:MT-CO2
3817:MT-CO1
3797:MT-CYB
3777:MT-ND6
3772:MT-ND5
3762:MT-ND4
3757:MT-ND3
3752:MT-ND2
3747:MT-ND1
3520:Matrix
3052:COQ10B
3047:COQ10A
2708:(MeSH)
2606:
2579:788795
2577:
2544:597492
2542:
2503:
2462:
2454:
2419:
2411:
2376:
2368:
2333:
2325:
2283:
2242:
2232:
2193:
2149:
2139:
2097:
2089:
2054:
2021:241101
2019:
1990:
1967:
1932:
1922:
1888:
1847:
1812:
1804:
1778:Nature
1761:
1718:
1595:UQCR10
1583:UQCRC1
1577:UQCRC2
1537:MT-CYB
1435:oxygen
1391:oxygen
1338:site.
1165:1 x Q
1155:2 x QH
1008:UQCR11
984:UQCR10
852:UQCRC2
821:UQCRC1
731:MT-CYB
611:-type
599:. The
491:type.
489:Rieske
296:PubMed
278:Search
264:AmiGO
252:PDBsum
192:ExPASy
180:BRENDA
168:IntEnz
139:EC no.
70:SUPFAM
48:Symbol
4333:Types
4128:Other
4045:1.10)
3977:MT-TY
3972:MT-TW
3967:MT-TV
3962:MT-TT
3947:MT-TR
3942:MT-TQ
3937:MT-TP
3932:MT-TN
3927:MT-TM
3912:MT-TK
3907:MT-TI
3902:MT-TH
3897:MT-TG
3892:MT-TF
3887:MT-TE
3882:MT-TD
3877:MT-TC
3872:MT-TA
3668:PMPCB
3655:ALDH2
3477:other
3171:Other
3137:TC 3D
3071:Other
3062:PDSS2
3057:PDSS1
2767:Cycle
2460:S2CID
2417:S2CID
2374:S2CID
2331:S2CID
2303:(PDF)
2095:S2CID
1810:S2CID
1618:BCS1L
1601:TTC19
1571:UQCRH
1565:UQCRB
1541:mtDNA
1373:Also
1271:Heme.
1220:Heme.
1169:to QH
936:UQCRQ
912:UQCRB
888:UQCRH
613:hemes
228:PRIAM
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66:SCOPe
57:SCOP2
51:(N/A)
4425:list
4418:EC7
4412:list
4405:EC6
4399:list
4392:EC5
4386:list
4379:EC4
4373:list
4366:EC3
4360:list
4353:EC2
4347:list
4340:EC1
3863:tRNA
3042:COQ9
3037:COQ7
3032:COQ6
3027:COQ5
3022:COQ4
3017:COQ3
3012:COQ2
2868:and
2805:SDHA
2716:OMIM
2604:PMID
2575:PMID
2540:PMID
2501:PMID
2452:PMID
2409:PMID
2366:PMID
2323:PMID
2281:PMID
2240:PMID
2191:PMID
2147:PMID
2087:PMID
2052:PMID
2017:PMID
1988:ISBN
1965:PMID
1930:PMID
1920:ISBN
1886:PMID
1845:PMID
1802:PMID
1759:PMID
1716:PMID
1692:1ntz
1589:UQCR
1553:CYCS
1547:CYC1
1322:and
1162:to Q
1016:Pfam
992:Pfam
968:Pfam
944:Pfam
920:Pfam
896:Pfam
867:Pfam
860:Pfam
836:Pfam
829:Pfam
800:Pfam
793:Pfam
763:Pfam
755:CYC1
739:Pfam
712:Pfam
696:No.
670:1L0L
660:1KYO
622:and
499:The
449:are
324:The
308:NCBI
249:PDBe
204:KEGG
106:3cx5
77:TCDB
62:1be3
3145:ETC
2918:to
2899:to
2880:to
2835:to
2714:on
2669:at
2596:doi
2565:doi
2561:456
2532:doi
2528:462
2491:doi
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2444:doi
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2358:doi
2315:doi
2271:doi
2230:PMC
2222:doi
2218:353
2181:doi
2177:279
2137:PMC
2129:doi
2121:AGE
2079:doi
2044:doi
1957:doi
1912:doi
1876:doi
1872:261
1837:doi
1833:134
1794:doi
1782:392
1751:doi
1739:281
1708:doi
1688:PDB
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1370:).
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1093:out
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656:PDB
284:PMC
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