585:). The architecture of the hydrophobic region of complex I shows multiple proton transporters that are mechanically interlinked. The three central components believed to contribute to this long-range conformational change event are the pH-coupled N2 iron-sulfur cluster, the quinone reduction, and the transmembrane helix subunits of the membrane arm. Transduction of conformational changes to drive the transmembrane transporters linked by a 'connecting rod' during the reduction of ubiquinone can account for two or three of the four protons pumped per NADH oxidized. The remaining proton must be pumped by direct coupling at the ubiquinone-binding site. It is proposed that direct and indirect coupling mechanisms account for the pumping of the four protons.
684:(EPR) spectra and double electron-electron resonance (DEER) to determine the path of electron transfer through the iron-sulfur complexes, which are located in the hydrophilic domain. Seven of these clusters form a chain from the flavin to the quinone binding sites; the eighth cluster is located on the other side of the flavin, and its function is unknown. The EPR and DEER results suggest an alternating or âroller-coasterâ potential energy profile for the electron transfer between the active sites and along the iron-sulfur clusters, which can optimize the rate of electron travel and allow efficient energy conversion in complex I.
2407:
disorder showed increased protein oxidation and nitration in their prefrontal cortex. These results suggest that future studies should target complex I for potential therapeutic studies for bipolar disorder. Similarly, Moran et al. (2010) found that patients with severe complex I deficiency showed decreased oxygen consumption rates and slower growth rates. However, they found that mutations in different genes in complex I lead to different phenotypes, thereby explaining the variations of pathophysiological manifestations of complex I deficiency.
31:
2240:
of idle enzyme to elevated, but physiological temperatures (>30 °C) in the absence of substrate, the enzyme converts to the D-form. This form is catalytically incompetent but can be activated by the slow reaction (k~4 min) of NADH oxidation with subsequent ubiquinone reduction. After one or several turnovers the enzyme becomes active and can catalyse physiological NADH:ubiquinone reaction at a much higher rate (k~10 min). In the presence of divalent cations (Mg, Ca), or at alkaline pH the activation takes much longer.
7538:
362:
354:
374:
2239:
The catalytic properties of eukaryotic complex I are not simple. Two catalytically and structurally distinct forms exist in any given preparation of the enzyme: one is the fully competent, so-called âactiveâ A-form and the other is the catalytically silent, dormant, âinactiveâ, D-form. After exposure
676:
Complex I contains a ubiquinone binding pocket at the interface of the 49-kDa and PSST subunits. Close to iron-sulfur cluster N2, the proposed immediate electron donor for ubiquinone, a highly conserved tyrosine constitutes a critical element of the quinone reduction site. A possible quinone exchange
672:
Three of the conserved, membrane-bound subunits in NADH dehydrogenase are related to each other, and to Mrp sodium-proton antiporters. Structural analysis of two prokaryotic complexes I revealed that the three subunits each contain fourteen transmembrane helices that overlay in structural alignments:
563:
The equilibrium dynamics of
Complex I are primarily driven by the quinone redox cycle. In conditions of high proton motive force (and accordingly, a ubiquinol-concentrated pool), the enzyme runs in the reverse direction. Ubiquinol is oxidized to ubiquinone, and the resulting released protons reduce
559:
The proposed pathway for electron transport prior to ubiquinone reduction is as follows: NADH â FMN â N3 â N1b â N4 â N5 â N6a â N6b â N2 â Q, where Nx is a labelling convention for iron sulfur clusters. The high reduction potential of the N2 cluster and the relative proximity of the other clusters
472:
The reaction can be reversed â referred to as aerobic succinate-supported NAD reduction by ubiquinol â in the presence of a high membrane potential, but the exact catalytic mechanism remains unknown. Driving force of this reaction is a potential across the membrane which can be maintained either by
2406:
Recent studies have examined other roles of complex I activity in the brain. Andreazza et al. (2010) found that the level of complex I activity was significantly decreased in patients with bipolar disorder, but not in patients with depression or schizophrenia. They found that patients with bipolar
2410:
Exposure to pesticides can also inhibit complex I and cause disease symptoms. For example, chronic exposure to low levels of dichlorvos, an organophosphate used as a pesticide, has been shown to cause liver dysfunction. This occurs because dichlorvos alters complex I and II activity levels, which
2316:
in mammalian mitochondria) pass through complex I to reduce NAD to NADH, driven by the inner mitochondrial membrane potential electric potential. Although it is not precisely known under what pathological conditions reverse-electron transfer would occur in vivo, in vitro experiments indicate that
2380:
Although the exact etiology of
Parkinson's disease is unclear, it is likely that mitochondrial dysfunction, along with proteasome inhibition and environmental toxins, may play a large role. In fact, the inhibition of complex I has been shown to cause the production of peroxides and a decrease in
2299:
During reverse electron transfer, complex I might be the most important site of superoxide production within mitochondria, with around 3-4% of electrons being diverted to superoxide formation. Reverse electron transfer, the process by which electrons from the reduced ubiquinol pool (supplied by
2192:
Despite more than 50 years of study of complex I, no inhibitors blocking the electron flow inside the enzyme have been found. Hydrophobic inhibitors like rotenone or piericidin most likely disrupt the electron transfer between the terminal FeS cluster N2 and ubiquinone. It has been shown that
664:
operon, and are homologous to mitochondrial complex I subunits. The antiporter-like subunits NuoL/M/N each contains 14 conserved transmembrane (TM) helices. Two of them are discontinuous, but subunit NuoL contains a 110 Ă
long amphipathic α-helix, spanning the entire length of the domain. The
2389:
Brain ischemia/reperfusion injury is mediated via complex I impairment. Recently it was found that oxygen deprivation leads to conditions in which mitochondrial complex I lose its natural cofactor, flavin mononucleotide (FMN) and become inactive. When oxygen is present the enzyme catalyzes a
677:
path leads from cluster N2 to the N-terminal beta-sheet of the 49-kDa subunit. All 45 subunits of the bovine NDHI have been sequenced. Each complex contains noncovalently bound FMN, coenzyme Q and several iron-sulfur centers. The bacterial NDHs have 8-9 iron-sulfur centers.
2188:
are even more potent inhibitors of complex I. They cross-link to the ND2 subunit, which suggests that ND2 is essential for quinone-binding. Rolliniastatin-2, an acetogenin, is the first complex I inhibitor found that does not share the same binding site as rotenone.
332:
This enzyme is essential for the normal functioning of cells, and mutations in its subunits lead to a wide range of inherited neuromuscular and metabolic disorders. Defects in this enzyme are responsible for the development of several pathological processes such as
445:, showing that in the tested conditions, the coupling ion is H. Na transport in the opposite direction was observed, and although Na was not necessary for the catalytic or proton transport activities, its presence increased the latter. H was translocated by the
620:
mechanism (Na/H swap) has been proposed using evidence of conserved Asp residues in the membrane arm. The presence of Lys, Glu, and His residues enable for proton gating (a protonation followed by deprotonation event across the membrane) driven by the
34:
NADH Dehydrogenase
Mechanism: 1. The seven primary iron sulfur centers serve to carry electrons from the site of NADH dehydration to ubiquinone. Note that N7 is not found in eukaryotes. 2. There is a reduction of ubiquinone (CoQ) to ubiquinol
7561:
2247:(270 kJ/mol) of the deactivation process indicates the occurrence of major conformational changes in the organisation of the complex I. However, until now, the only conformational difference observed between these two forms is the number of
5152:
Muller FL, Liu Y, Abdul-Ghani MA, Lustgarten MS, Bhattacharya A, Jang YC, Van Remmen H (January 2008). "High rates of superoxide production in skeletal-muscle mitochondria respiring on both complex I- and complex II-linked substrates".
655:
The structure is an "L" shape with a long membrane domain (with around 60 trans-membrane helices) and a hydrophilic (or peripheral) domain, which includes all the known redox centres and the NADH binding site. All thirteen of the
2385:
activity, which may lead to
Parkinson's disease. Additionally, Esteves et al. (2010) found that cell lines with Parkinson's disease show increased proton leakage in complex I, which causes decreased maximum respiratory capacity.
2398:
so that fraction of electrons from succinate is directed upstream to FMN of complex I. Reverse electron transfer results in a reduction of complex I FMN, increased generation of ROS, followed by a loss of the reduced cofactor
2204:
of NADH oxidation â by binding to the enzyme at the nucleotide binding site. Both hydrophilic NADH and hydrophobic ubiquinone analogs act at the beginning and the end of the internal electron-transport pathway, respectively.
601:, providing evidence for the "single stroke" H translocation mechanism (i.e. all four protons move across the membrane at the same time). Alternative theories suggest a "two stroke mechanism" where each reduction step (
572:
The coupling of proton translocation and electron transport in
Complex I is currently proposed as being indirect (long range conformational changes) as opposed to direct (redox intermediates in the hydrogen pumps as in
3099:
Petrussa E, Bertolini A, Casolo V, KrajnĂĄkovĂĄ J, MacrĂŹ F, Vianello A (December 2009). "Mitochondrial bioenergetics linked to the manifestation of programmed cell death during somatic embryogenesis of Abies alba".
2268:. It is likely that transition from the active to the inactive form of complex I takes place during pathological conditions when the turnover of the enzyme is limited at physiological temperatures, such as during
588:
The N2 cluster's proximity to a nearby cysteine residue results in a conformational change upon reduction in the nearby helices, leading to small but important changes in the overall protein conformation. Further
2332:
Superoxide is a reactive oxygen species that contributes to cellular oxidative stress and is linked to neuromuscular diseases and aging. NADH dehydrogenase produces superoxide by transferring one electron from
2259:
irreversibly blocks critical cysteine residues, abolishing the ability of the enzyme to respond to activation, thus inactivating it irreversibly. The A-form of complex I is insensitive to sulfhydryl reagents.
2263:
It was found that these conformational changes may have a very important physiological significance. The inactive, but not the active form of complex I was susceptible to inhibition by nitrosothiols and
2170:). There have been reports of the indigenous people of French Guiana using rotenone-containing plants to fish - due to its ichthyotoxic effect - as early as the 17th century. Rotenone binds to the
6351:
5975:
4461:
Watabe M, Nakaki T (October 2008). "Mitochondrial complex I inhibitor rotenone inhibits and redistributes vesicular monoamine transporter 2 via nitration in human dopaminergic SH-SY5Y cells".
4190:
Saada A, Vogel RO, Hoefs SJ, van den Brand MA, Wessels HJ, Willems PH, Venselaar H, Shaag A, Barghuti F, Reish O, Shohat M, Huynen MA, Smeitink JA, van den Heuvel LP, Nijtmans LG (June 2009).
2341:). The radical flavin leftover is unstable, and transfers the remaining electron to the iron-sulfur centers. It is the ratio of NADH to NAD that determines the rate of superoxide formation.
465:
enzyme is completely Na independent. It is also possible that another transporter catalyzes the uptake of Na. Complex I energy transduction by proton pumping may not be exclusive to the
2296:), through at least two different pathways. During forward electron transfer, only very small amounts of superoxide are produced (probably less than 0.1% of the overall electron flow).
7565:
2212:
has been shown to induce a mild and transient inhibition of the mitochondrial respiratory chain complex I, and this inhibition appears to play a key role in its mechanism of action.
637:, the enzyme contains 44 separate water-soluble peripheral membrane proteins, which are anchored to the integral membrane constituents. Of particular functional importance are the
5694:
Binukumar BK, Bal A, Kandimalla R, Sunkaria A, Gill KD (April 2010). "Mitochondrial energy metabolism impairment and liver dysfunction following chronic exposure to dichlorvos".
2447:
serves to maintain photosynthesis in stressful situations. This makes it at least partially dispensable in favorable conditions. It is evident that angiosperm lineages without
2390:
physiological reaction of NADH oxidation by ubiquinone, supplying electrons downstream of the respiratory chain (complexes III and IV). Ischemia leads to dramatic increase of
469:
enzyme. The Na/H antiport activity seems not to be a general property of complex I. However, the existence of Na-translocating activity of the complex I is still in question.
3594:
Hunte C, Screpanti E, Venturi M, Rimon A, Padan E, Michel H (June 2005). "Structure of a Na+/H+ antiporter and insights into mechanism of action and regulation by pH".
6452:
6344:
6314:
296:
5968:
2403:) and impairment of mitochondria energy production. The FMN loss by complex I and I/R injury can be alleviated by the administration of FMN precursor, riboflavin.
125:
113:
4631:"Mitochondrial complex I inhibition triggers NAD+-independent glucose oxidation via successive NADPH formation, "futile" fatty acid cycling, and FADH2 oxidation"
6248:
315:
3957:
GabaldĂłn T, Rainey D, Huynen MA (May 2005). "Tracing the evolution of a large protein complex in the eukaryotes, NADH:ubiquinone oxidoreductase (Complex I)".
560:
in the chain enable efficient electron transfer over long distance in the protein (with transfer rates from NADH to N2 iron-sulfur cluster of about 100 ÎŒs).
6337:
4053:"Mitochondrial NADH:ubiquinone oxidoreductase (complex I) in eukaryotes: a highly conserved subunit composition highlighted by mining of protein databases"
5239:"Production of reactive oxygen species by complex I (NADH:ubiquinone oxidoreductase) from Escherichia coli and comparison to the enzyme from mitochondria"
6933:
5961:
5008:
Hansford RG, Hogue BA, Mildaziene V (February 1997). "Dependence of H2O2 formation by rat heart mitochondria on substrate availability and donor age".
4242:
Balsa, Eduardo; Marco, Ricardo; Perales-Clemente, Ester; Szklarczyk, Radek; Calvo, Enrique; LandĂĄzuri, Manuel O.; EnrĂquez, JosĂ© Antonio (2012-09-05).
39:). 3. The energy from the redox reaction results in conformational change allowing hydrogen ions to pass through four transmembrane helix channels.
6928:
7019:
6601:
6388:
6219:
578:
390:
5843:
4192:"Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease"
593:
studies of the electron transfer have demonstrated that most of the energy that is released during the subsequent CoQ reduction is on the final
6687:
5105:"Reverse electron transfer results in a loss of flavin from mitochondrial complex I: Potential mechanism for brain ischemia reperfusion injury"
2362:
3204:
519:
All redox reactions take place in the hydrophilic domain of complex I. NADH initially binds to complex I, and transfers two electrons to the
6496:
5614:"Mitochondrial complex I activity and oxidative damage to mitochondrial proteins in the prefrontal cortex of patients with bipolar disorder"
6445:
3918:"Higher plant-like subunit composition of mitochondrial complex I from Chlamydomonas reinhardtii: 31 conserved components among eukaryotes"
535:(ubiquinone). This electron flow changes the redox state of the protein, inducing conformational changes of the protein which alters the p
5053:"The dependence of brain mitochondria reactive oxygen species production on oxygen level is linear, except when inhibited by antimycin A"
7596:
6368:
5917:
5429:"Mitochondrial respiration and respiration-associated proteins in cell lines created through Parkinson's subject mitochondrial transfer"
616:
localized to the membrane domain interacts with negatively charged residues in the membrane arm, stabilizing conformational changes. An
6787:
6722:
6101:
6020:
2309:
2305:
4629:
Abrosimov, Roman; Baeken, Marius W.; Hauf, Samuel; Wittig, Ilka; Hajieva, Parvana; Perrone, Carmen E.; Moosmann, Bernd (2024-01-25).
4418:"Natural substances (acetogenins) from the family Annonaceae are powerful inhibitors of mitochondrial NADH dehydrogenase (Complex I)"
7257:
6732:
6557:
3662:
3263:"Mössbauer spectroscopy on respiratory complex I: the iron-sulfur cluster ensemble in the NADH-reduced enzyme is partially oxidized"
2419:
A proton-pumping, ubiquinone-using NADH dehydrogenase complex, homologous to complex I, is found in the chloroplast genomes of most
3545:
3994:"Direct assignment of EPR spectra to structurally defined iron-sulfur clusters in complex I by double electron-electron resonance"
7058:
6856:
6491:
2443:. The purpose of this complex is originally cryptic as chloroplasts do not participate in respiration, but now it is known that
308:
7225:
7053:
6717:
6438:
681:
590:
7048:
6834:
6792:
6258:
6088:
6076:
6025:
5902:
2427:. This complex is inherited from the original symbiosis from cyanobacteria, but has been lost in most eukaryotic algae, some
235:
7413:
5791:
5427:
Esteves AR, Lu J, Rodova M, Onyango I, Lezi E, Dubinsky R, Lyons KE, Pahwa R, Burns JM, Cardoso SM, Swerdlow RH (May 2010).
4141:
Dunning CJ, McKenzie M, Sugiana C, Lazarou M, Silke J, Connelly A, Fletcher JM, Kirby DM, Thorburn DR, Ryan MT (July 2007).
3468:(August 2007). "Single particle analysis confirms distal location of subunits NuoL and NuoM in Escherichia coli complex I".
259:
389:. There are three energy-transducing enzymes in the electron transport chain - NADH:ubiquinone oxidoreductase (complex I),
7581:
6097:
5795:
7528:
5513:"Critical Role of Flavin and Glutathione in Complex I-Mediated Bioenergetic Failure in Brain Ischemia/Reperfusion Injury"
6638:
6580:
6470:
6084:
2313:
2197:
5190:"Krebs cycle metabolites and preferential succinate oxidation following neonatal hypoxic-ischemic brain injury in mice"
6692:
5836:
528:
6329:
5280:"The mechanism of superoxide production by NADH:ubiquinone oxidoreductase (complex I) from bovine heart mitochondria"
4913:
Moncada S, Erusalimsky JD (March 2002). "Does nitric oxide modulate mitochondrial energy generation and apoptosis?".
2918:"-->H+/2e- stoichiometry in NADH-quinone reductase reactions catalyzed by bovine heart submitochondrial particles"
451:
complex I, but in this case, H transport was not influenced by Na, and Na transport was not observed. Possibly, the
6866:
6861:
6665:
6531:
7398:
4143:"Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease"
2193:
long-term systemic inhibition of complex I by rotenone can induce selective degeneration of dopaminergic neurons.
7514:
7501:
7488:
7475:
7462:
7449:
7436:
6591:
6414:
6185:
5882:
5877:
5653:
MorĂĄn M, Rivera H, SĂĄnchez-AragĂł M, BlĂĄzquez A, Merinero B, Ugalde C, Arenas J, Cuezva JM, MartĂn MA (May 2010).
2251:
residues exposed at the surface of the enzyme. Treatment of the D-form of complex I with the sulfhydryl reagents
2093:
531:. The electrons are then transferred through the FMN via a series of iron-sulfur (Fe-S) clusters, and finally to
428:
7408:
4817:"Attenuation of oxidative damage by targeting mitochondrial complex I in neonatal hypoxic-ischemic brain injury"
502:
As a result of a two NADH molecule being oxidized to NAD+, three molecules of ATP can be produced by
Complex V (
7362:
7305:
6782:
6660:
6180:
6140:
6015:
5815:
1201:
1157:
1127:
1090:
1060:
1023:
993:
963:
957:
927:
921:
891:
885:
855:
849:
819:
813:
783:
777:
747:
741:
447:
386:
253:
158:
70:
47:
3360:"Evidence for two sites of superoxide production by mitochondrial NADH-ubiquinone oxidoreductase (complex I)"
7310:
6829:
6616:
6205:
6121:
6032:
5912:
5897:
5892:
2301:
2285:
539:
values of ionizable side chain, and causes four hydrogen ions to be pumped out of the mitochondrial matrix.
455:
complex I has two energy coupling sites (one Na independent and the other
Nadependent), as observed for the
240:
3152:
Sazanov LA (June 2015). "A giant molecular proton pump: structure and mechanism of respiratory complex I".
2970:"Sodium influence on energy transduction by complexes I from Escherichia coli and Paracoccus denitrificans"
2836:"Two protons are pumped from the mitochondrial matrix per electron transferred between NADH and ubiquinone"
2488:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4, 9kDa - recently described to be part of complex IV
441:
complex I (NADH dehydrogenase) is capable of proton translocation in the same direction to the established
6824:
6481:
6409:
6164:
6113:
6080:
5872:
5829:
5339:"Superoxide radical formation by pure complex I (NADH:ubiquinone oxidoreductase) from Yarrowia lipolytica"
2366:
2350:
432:
30:
4864:
Stepanova A, Konrad C, Guerrero-Castillo S, Manfredi G, Vannucci S, Arnold S, Galkin A (September 2019).
4293:"Essential structural factors of annonaceous acetogenins as potent inhibitors of mitochondrial complex I"
3719:
Balsa E, Marco R, Perales-Clemente E, Szklarczyk R, Calvo E, LandĂĄzuri MO, EnrĂquez JA (September 2012).
2877:"The proton pumping stoichiometry of purified mitochondrial complex I reconstituted into proteoliposomes"
7331:
7250:
7198:
6938:
6814:
6650:
6628:
6058:
5942:
5867:
5811:
5563:
Stepanova A, Sosunov S, Niatsetskaya Z, Konrad C, Starkov AA, Manfredi G, et al. (September 2019).
4506:"A competitive inhibition of the mitochondrial NADH-ubiquinone oxidoreductase (complex I) by ADP-ribose"
2395:
2201:
520:
481:
342:
320:
7403:
4094:"A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy"
228:
2329:
concentrations are low. This can take place during tissue ischaemia, when oxygen delivery is blocked.
7039:
6945:
6802:
6751:
6697:
6393:
6229:
6159:
6047:
5291:
4005:
3833:
3776:
3603:
2104:
649:
582:
457:
394:
397:(complex IV). Complex I is the largest and most complicated enzyme of the electron transport chain.
7586:
7367:
6309:
645:
137:
5468:
Galkin A (November 2019). "Brain
Ischemia/Reperfusion Injury and Mitochondrial Complex I Damage".
4369:"The ND2 subunit is labeled by a photoaffinity analogue of asimicin, a potent complex I inhibitor"
256:
7300:
6969:
6737:
6611:
6383:
6200:
5922:
5493:
5033:
4938:
4486:
3857:
3802:
3627:
3576:
3177:
3125:
2947:
334:
180:
5565:"Redox-Dependent Loss of Flavin by Mitochondrial Complex I in Brain Ischemia/Reperfusion Injury"
5188:
Sahni PV, Zhang J, Sosunov S, Galkin A, Niatsetskaya Z, Starkov A, et al. (February 2018).
3546:"Structural biology. Mechanistic insight from the crystal structure of mitochondrial complex I"
17:
7591:
6959:
6762:
6543:
5988:
5937:
5932:
5766:
5711:
5676:
5635:
5594:
5542:
5485:
5450:
5409:
5360:
5319:
5260:
5219:
5170:
5134:
5082:
5025:
4990:
4930:
4895:
4846:
4815:
Kim M, Stepanova A, Niatsetskaya Z, Sosunov S, Arndt S, Murphy MP, et al. (August 2018).
4797:
4756:
4707:
4648:
4611:
4576:
4527:
4478:
4443:
4398:
4349:
4314:
4273:
4265:
4221:
4172:
4123:
4074:
4033:
3974:
3939:
3898:
3849:
3794:
3742:
3701:
3658:
3619:
3568:
3526:
3485:
3446:
3391:
3340:
3292:
3243:
3200:
3169:
3117:
3081:
3040:
2991:
2939:
2898:
2857:
2816:
2293:
2269:
2244:
247:
4367:
Nakamaru-Ogiso E, Han H, Matsuno-Yagi A, Keinan E, Sinha SC, Yagi T, Ohnishi T (March 2010).
3319:"The coupling mechanism of respiratory complex I - a structural and evolutionary perspective"
3011:"Redox-dependent change of nucleotide affinity to the active site of the mammalian complex I"
2812:
7346:
7341:
7315:
7243:
6772:
6565:
6283:
6278:
6151:
6005:
5786:
5756:
5746:
5703:
5666:
5625:
5584:
5576:
5532:
5524:
5477:
5440:
5399:
5391:
5350:
5309:
5299:
5250:
5209:
5201:
5162:
5124:
5116:
5072:
5064:
5017:
4980:
4972:
4922:
4885:
4877:
4836:
4828:
4787:
4746:
4738:
4697:
4689:
4656:
4638:
4603:
4566:
4558:
4517:
4470:
4433:
4425:
4388:
4380:
4341:
4304:
4255:
4211:
4203:
4162:
4154:
4113:
4105:
4064:
4023:
4013:
3966:
3929:
3888:
3841:
3784:
3732:
3691:
3654:
3611:
3560:
3516:
3477:
3436:
3426:
3381:
3371:
3330:
3282:
3274:
3233:
3161:
3109:
3071:
3030:
3022:
2981:
2929:
2888:
2847:
2808:
2252:
641:
437:
3544:
Zickermann V, Wirth C, Nasiri H, Siegmund K, Schwalbe H, Hunte C, Brandt U (January 2015).
2411:
leads to decreased mitochondrial electron transfer activities and decreased ATP synthesis.
7393:
7377:
7290:
6677:
6570:
6360:
2125:
673:
the translocation of three protons may be coordinated by a lateral helix connecting them.
496:
442:
216:
66:
3916:
Cardol P, Vanrobaeys F, Devreese B, Van
Beeumen J, Matagne RF, Remacle C (October 2004).
480:. In fact, there has been shown to be a correlation between mitochondrial activities and
5953:
5295:
4832:
4661:
4009:
3837:
3780:
3765:"Structure of the hydrophilic domain of respiratory complex I from Thermus thermophilus"
3607:
192:
7542:
7431:
7372:
5787:
Institute of
Science and Technology Austria (ISTA): Sazanov Group MRC MBU Sazanov group
5761:
5730:
5655:"Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts"
5589:
5564:
5537:
5512:
5511:
Kahl A, Stepanova A, Konrad C, Anderson C, Manfredi G, Zhou P, et al. (May 2018).
5404:
5379:
5314:
5279:
5214:
5189:
5129:
5104:
5077:
5052:
4985:
4960:
4890:
4865:
4841:
4816:
4751:
4726:
4702:
4677:
4571:
4438:
4417:
4393:
4368:
4216:
4191:
4167:
4142:
4118:
4093:
4028:
3993:
3821:
3760:
3465:
3441:
3414:
3410:
3386:
3359:
3314:
3287:
3262:
3035:
3010:
2354:
2216:
291:
163:
118:
5630:
5613:
4866:"Deactivation of mitochondrial complex I after hypoxia-ischemia in the immature brain"
4522:
4505:
4309:
4292:
3238:
3221:
3009:
Grivennikova VG, Kotlyar AB, Karliner JS, Cecchini G, Vinogradov AD (September 2007).
2934:
2917:
271:
7575:
7336:
7295:
6461:
5927:
5497:
5445:
5428:
4345:
2852:
2835:
2391:
2265:
2141:
266:
4942:
4594:
Nadanaciva S, Will Y (2011). "New insights in drug-induced mitochondrial toxicity".
4546:
3992:
Roessler MM, King MS, Robinson AJ, Armstrong FA, Harmer J, Hirst J (February 2010).
3580:
3181:
3129:
2451:
do not last long from their young ages, but how gymnosperms survive on land without
2073:
2051:
2029:
2007:
1980:
1937:
1913:
1889:
1865:
1841:
1817:
1793:
1769:
1745:
1721:
1697:
1673:
1649:
1625:
1601:
1577:
1553:
1529:
1505:
1481:
1457:
1433:
1409:
1385:
1361:
1337:
1313:
1289:
1265:
1241:
1212:
1182:
1175:
1168:
1138:
1108:
1101:
1071:
1041:
1034:
1004:
974:
938:
902:
866:
830:
794:
758:
7285:
7094:
7069:
6727:
6606:
6519:
6224:
6210:
6132:
5887:
5856:
5037:
4727:"Ischemic A/D transition of mitochondrial complex I and its role in ROS generation"
4545:
Viollet B, Guigas B, Sanz Garcia N, Leclerc J, Foretz M, Andreelli F (March 2012).
4490:
3861:
3806:
3647:
3631:
2951:
2692:â NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase)
2686:â NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase)
2680:â NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase)
2674:â NADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase)
2668:â NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
2662:â NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase)
2656:â NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase)
2650:â NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase)
2322:
2273:
2175:
2155:
638:
532:
503:
86:
82:
4384:
3678:
Carroll J, Fearnley IM, Skehel JM, Shannon RJ, Hirst J, Walker JE (October 2006).
2365:.There is some evidence that complex I defects may play a role in the etiology of
666:
527:. The electron acceptor â the isoalloxazine ring â of FMN is identical to that of
142:
130:
7556:
5806:
5671:
5654:
5528:
4776:"S-nitrosation of mitochondrial complex I depends on its structural conformation"
4742:
4693:
4069:
4052:
3934:
3917:
3893:
3876:
3521:
3504:
3335:
3318:
2986:
2969:
2893:
2876:
7509:
7444:
7280:
6364:
5395:
4678:"Characterisation of the active/de-active transition of mitochondrial complex I"
4291:
Miyoshi H, Ohshima M, Shimada H, Akagi T, Iwamura H, McLaughlin JL (July 1998).
2436:
2370:
2357:. Point mutations in various complex I subunits derived from mitochondrial DNA (
2146:
602:
598:
420:
275:
7537:
5284:
Proceedings of the National Academy of Sciences of the United States of America
4643:
4607:
4260:
4243:
4207:
3998:
Proceedings of the National Academy of Sciences of the United States of America
3737:
3720:
2560:
NDUFAF4 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 4
2557:
NDUFAF3 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 3
2554:
NDUFAF2 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 2
633:
NADH:ubiquinone oxidoreductase is the largest of the respiratory complexes. In
6846:
6508:
6068:
5984:
5907:
5707:
5481:
5051:
Stepanova A, Konrad C, Manfredi G, Springett R, Ten V, Galkin A (March 2019).
5021:
3970:
3481:
3195:
Voet DJ, Voet GJ, Pratt CW (2008). "Chapter 18, Mitochondrial ATP synthesis".
3113:
2799:
Brandt U (2006). "Energy converting NADH:quinone oxidoreductase (complex I)".
2440:
2428:
2420:
2382:
2374:
2289:
2224:
2185:
2181:
2171:
2129:
fruit) is the most potent known inhibitor of NADH dehydrogenase (ubiquinone) (
2120:
2116:
617:
540:
5120:
4881:
4652:
4630:
4269:
4244:"NDUFA4 is a subunit of complex IV of the mammalian electron transport chain"
4158:
3721:"NDUFA4 is a subunit of complex IV of the mammalian electron transport chain"
2463:
The following is a list of humans genes that encode components of complex I:
492:
7483:
7457:
6777:
6010:
5304:
5103:
Stepanova A, Kahl A, Konrad C, Ten V, Starkov AS, Galkin A (December 2017).
4018:
3789:
3764:
3564:
3431:
3376:
2318:
2220:
2209:
2178:, another potent inhibitor with a close structural homologue to ubiquinone.
2160:
613:
606:
594:
544:
477:
5770:
5715:
5680:
5639:
5598:
5546:
5489:
5454:
5413:
5364:
5355:
5338:
5323:
5264:
5223:
5174:
5138:
5086:
4994:
4934:
4899:
4850:
4801:
4792:
4775:
4760:
4711:
4615:
4580:
4482:
4474:
4402:
4277:
4225:
4176:
4127:
4078:
4037:
3978:
3943:
3902:
3853:
3798:
3746:
3705:
3696:
3679:
3623:
3572:
3530:
3489:
3450:
3395:
3344:
3296:
3173:
3121:
3085:
3044:
2995:
2943:
2902:
2820:
2545:
NDUFAB1 â NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1, 8kDa
2394:
level. In the presence of succinate mitochondria catalyze reverse electron
2136:=1.2 nM, stronger than rotenone). The best-known inhibitor of complex I is
5751:
5580:
5029:
4531:
4447:
4353:
4318:
3247:
3076:
3059:
2861:
361:
353:
6923:
6797:
6430:
6419:
6042:
5821:
3505:"A two-state stabilization-change mechanism for proton-pumping complex I"
2530:
NDUFA11 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 11, 14.7kDa
2248:
2166:
2137:
1926:
74:
5205:
3845:
3615:
966:
930:
894:
858:
822:
786:
750:
73:
of many organisms from bacteria to humans. It catalyzes the transfer of
7083:
7078:
6998:
5852:
5166:
4976:
4562:
4416:
Degli Esposti M, Ghelli A, Ratta M, Cortes D, Estornell E (July 1994).
2747:
2619:
2613:
2551:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 1
2548:
2539:
2533:
2524:
2509:
NDUFA7 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa
2494:
NDUFA4L2 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2
2228:
1902:
1662:
1566:
1494:
1470:
1326:
1254:
1204:
1160:
1130:
1121:
1093:
1063:
1026:
996:
960:
924:
888:
852:
816:
780:
744:
710:
609:) results in a stroke of two protons entering the intermembrane space.
223:
204:
50:
5255:
5238:
5098:
5096:
5068:
4429:
3278:
3026:
660:
proteins, which comprise NADH dehydrogenase I, are encoded within the
473:
ATP-hydrolysis or by complexes III and IV during succinate oxidation.
7496:
7266:
7188:
7183:
7153:
7148:
7028:
7008:
7003:
6993:
6988:
6983:
6978:
5991:
4109:
2788:(6th ed.). New York: WH Freeman & Company. pp. 509â513.
2759:
2753:
2741:
2735:
2729:
2723:
2712:
2706:
2700:
2689:
2683:
2677:
2671:
2665:
2659:
2653:
2647:
2636:
2630:
2607:
2601:
2595:
2589:
2574:
2568:
2518:
2512:
2503:
2497:
2485:
2476:
2470:
2326:
2227:. Further, complex I inhibition was shown to trigger NAD-independent
2151:
2100:
1969:
1950:
1878:
1854:
1830:
1806:
1782:
1758:
1734:
1710:
1686:
1638:
1614:
1590:
1542:
1518:
1446:
1422:
1398:
1374:
1350:
1302:
1278:
1230:
1195:
1151:
1084:
1054:
1017:
987:
951:
915:
879:
843:
807:
771:
735:
700:
634:
338:
303:
199:
187:
175:
3165:
2586:
NDUFB5 â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa
2583:
NDUFB4 â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4, 15kDa
2580:
NDUFB3 â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 3, 12kDa
2491:
NDUFA4L â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like
2482:
NDUFA3 â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3, 9kDa
2140:(commonly used as an organic pesticide). Rotenone and rotenoids are
7557:"3.D.1 The H+ or Na+-translocating NADH Dehydrogenase (NDH) Family"
4926:
3060:"Mitochondrial complex I in the network of known and unknown facts"
2639:â NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2, 14.5kDa
2284:
Recent investigations suggest that complex I is a potent source of
7470:
7208:
7203:
7193:
7178:
7173:
7168:
7163:
7158:
7143:
7138:
7133:
7128:
7123:
7118:
7113:
7108:
7103:
6899:
6886:
6293:
6288:
2369:, perhaps because of reactive oxygen species (complex I can, like
2358:
696:
373:
3875:
Tocilescu MA, Zickermann V, Zwicker K, Brandt U (December 2010).
6273:
6268:
6263:
6253:
6243:
6036:
5659:
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
2633:â NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1, 6kDa
2622:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11, 17.3kDa
2256:
2144:
occurring in several genera of tropical plants such as Antonia (
2130:
2070:
2048:
2026:
2004:
1977:
1934:
1910:
1907:
NADH dehydrogenase 1 alpha subcomplex subunit 10, mitochondrial
1886:
1862:
1838:
1814:
1790:
1766:
1742:
1718:
1694:
1670:
1646:
1622:
1598:
1574:
1550:
1526:
1502:
1478:
1454:
1430:
1406:
1382:
1358:
1334:
1310:
1286:
1262:
1238:
1209:
1179:
1172:
1165:
1135:
1105:
1098:
1068:
1038:
1031:
1001:
971:
935:
899:
863:
827:
791:
755:
716:
574:
424:
211:
78:
7239:
6434:
6333:
5957:
5825:
2527:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa
2473:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa
1499:
NADH dehydrogenase 1 beta subcomplex subunit 11, mitochondrial
1307:
NADH dehydrogenase 1 alpha subcomplex subunit 9, mitochondrial
2616:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10, 22kDa
2521:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa
2515:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa
2506:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa
2500:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5, 13kDa
1835:
NADH dehydrogenase 1 beta subcomplex subunit 5, mitochondrial
1739:
NADH dehydrogenase 1 beta subcomplex subunit 2, mitochondrial
1691:
NADH dehydrogenase 1 beta subcomplex subunit 8, mitochondrial
4547:"Cellular and molecular mechanisms of metformin: an overview"
3415:"Structural basis for the mechanism of respiratory complex I"
2610:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa
2604:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa
2598:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa
2592:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa
2479:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa
956:
NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial
7562:
Creative Commons Attribution-ShareAlike 3.0 Unported License
2577:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2, 8kDa
2571:â NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 1, 7kDa
2317:
this process can be a very potent source of superoxide when
5378:
Chou AP, Li S, Fitzmaurice AG, Bronstein JM (August 2010).
89:
membrane in eukaryotes or the plasma membrane of bacteria.
7560:, which is licensed in a way that permits reuse under the
7235:
5801:
4237:
4235:
3824:(May 2010). "The architecture of respiratory complex I".
2067:
NADH dehydrogenase 1 alpha subcomplex, assembly factor 4
2023:
NADH dehydrogenase 1 alpha subcomplex, assembly factor 2
2001:
NADH dehydrogenase 1 alpha subcomplex, assembly factor 1
884:
NADH dehydrogenase iron-sulfur protein 2, mitochondrial
848:
NADH dehydrogenase iron-sulfur protein 3, mitochondrial
776:
NADH dehydrogenase iron-sulfur protein 8, mitochondrial
740:
NADH dehydrogenase iron-sulfur protein 7, mitochondrial
5237:
EsterhĂĄzy D, King MS, Yakovlev G, Hirst J (March 2008).
3877:"Quinone binding and reduction by respiratory complex I"
3680:"Bovine complex I is a complex of 45 different subunits"
3653:(3rd ed.). New York: J. Wiley & Sons. pp.
2542:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13
2536:â NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12
2045:
NADH dehydrogenase 1 alpha subcomplex assembly factor 3
1283:
NADH dehydrogenase iron-sulfur protein 4, mitochondrial
1235:
NADH dehydrogenase iron-sulfur protein 6, mitochondrial
7551:
2750:- mitochondrially encoded NADH dehydrogenase subunit 4L
2715:â NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa
2709:â NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa
2703:â NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa
1931:
NADH dehydrogenase 1 alpha subcomplex subunit 4-like 2
5612:
Andreazza AC, Shao L, Wang JF, Young LT (April 2010).
5380:"Mechanisms of rotenone-induced proteasome inhibition"
4092:
Ogilvie I, Kennaway NG, Shoubridge EA (October 2005).
2916:
Galkin AS, Grivennikova VG, Vinogradov AD (May 1999).
2762:- mitochondrially encoded NADH dehydrogenase subunit 6
2756:- mitochondrially encoded NADH dehydrogenase subunit 5
2744:- mitochondrially encoded NADH dehydrogenase subunit 4
2738:- mitochondrially encoded NADH dehydrogenase subunit 3
2732:- mitochondrially encoded NADH dehydrogenase subunit 2
2726:- mitochondrially encoded NADH dehydrogenase subunit 1
2627:
NADH dehydrogenase (ubiquinone) 1, subcomplex unknown
7526:
4676:
Babot M, Birch A, Labarbuta P, Galkin A (July 2014).
3308:
3306:
648:(FeS). Of the 44 subunits, seven are encoded by the
7422:
7386:
7355:
7324:
7273:
7092:
7067:
7037:
7017:
6967:
6958:
6912:
6875:
6843:
6811:
6759:
6750:
6706:
6674:
6647:
6625:
6588:
6579:
6556:
6528:
6505:
6478:
6469:
6402:
6376:
6302:
6193:
6177:
6149:
6130:
6111:
6066:
6057:
5998:
4731:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
4682:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
4510:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
4297:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
4057:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
3922:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
3881:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
3509:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
3323:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
3226:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
2974:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
2881:
Biochimica et Biophysica Acta (BBA) - Bioenergetics
2720:mitochondrially encoded NADH dehydrogenase subunit
2467:NADH dehydrogenase (ubiquinone) 1 alpha subcomplex
523:(FMN) prosthetic group of the enzyme, creating FMNH
423:across the inner membrane per molecule of oxidized
314:
302:
290:
285:
265:
246:
234:
222:
210:
198:
186:
174:
169:
157:
152:
136:
124:
112:
104:
99:
94:
4961:"How mitochondria produce reactive oxygen species"
3646:
2565:NADH dehydrogenase (ubiquinone) 1 beta subcomplex
2276::oxygen ratio increases (i.e. metabolic hypoxia).
920:NADH dehydrogenase flavoprotein 1, mitochondrial
812:NADH dehydrogenase flavoprotein 2, mitochondrial
665:subunit, NuoL, is related to Na/ H antiporters of
85:(CoQ10) and translocates protons across the inner
5792:Interactive Molecular model of NADH dehydrogenase
2963:
2961:
2373:, leak electrons to oxygen, forming highly toxic
2349:Mutations in the subunits of complex I can cause
2219:associated with a variety of drugs, for instance
1571:NADH dehydrogenase 1 alpha subcomplex subunit 13
1475:NADH dehydrogenase 1 alpha subcomplex subunit 11
1259:NADH dehydrogenase 1 alpha subcomplex subunit 12
461:complex I, whereas the coupling mechanism of the
6315:Electron-transferring-flavoprotein dehydrogenase
5731:"On the Edge of Dispensability, the Chloroplast
3358:Treberg JR, Quinlan CL, Brand MD (August 2011).
3222:"Iron-sulfur clusters/semiquinones in complex I"
1811:NADH dehydrogenase 1 alpha subcomplex subunit 4
1787:NADH dehydrogenase 1 alpha subcomplex subunit 3
1763:NADH dehydrogenase 1 alpha subcomplex subunit 7
1667:NADH dehydrogenase 1 beta subcomplex subunit 10
1619:NADH dehydrogenase 1 alpha subcomplex subunit 8
1451:NADH dehydrogenase 1 alpha subcomplex subunit 6
1427:NADH dehydrogenase 1 alpha subcomplex subunit 5
1379:NADH dehydrogenase 1 alpha subcomplex subunit 1
1355:NADH dehydrogenase 1 alpha subcomplex subunit 2
489:Na-translocating NADH Dehydrogenase (NDH) Family
27:Protein complex involved in cellular respiration
6220:Complex III/Coenzyme Q - cytochrome c reductase
4954:
4952:
2697:NADH dehydrogenase (ubiquinone) flavoprotein 1
2215:Inhibition of complex I has been implicated in
1974:NADH dehydrogenase 1 beta subcomplex subunit 6
1883:NADH dehydrogenase 1 subunit C1, mitochondrial
1859:NADH dehydrogenase 1 beta subcomplex subunit 1
1643:NADH dehydrogenase 1 beta subcomplex subunit 9
1595:NADH dehydrogenase 1 beta subcomplex subunit 7
1547:NADH dehydrogenase 1 beta subcomplex subunit 4
1403:NADH dehydrogenase 1 beta subcomplex subunit 3
419:In this process, the complex translocates four
7251:
6446:
6345:
5969:
5837:
5109:Journal of Cerebral Blood Flow and Metabolism
4870:Journal of Cerebral Blood Flow and Metabolism
2875:Galkin A, Dröse S, Brandt U (December 2006).
2644:NADH dehydrogenase (ubiquinone) Fe-S protein
543:(CoQ) accepts two electrons to be reduced to
8:
4725:Dröse S, Stepanova A, Galkin A (July 2016).
3261:Bridges HR, Bill E, Hirst J (January 2012).
5739:International Journal of Molecular Sciences
4332:Moretti C, Grenand P (September 1982). "".
7258:
7244:
7236:
6964:
6934:Mitochondrial permeability transition pore
6916:
6756:
6585:
6475:
6453:
6439:
6431:
6352:
6338:
6330:
6190:
6063:
5976:
5962:
5954:
5844:
5830:
5822:
282:
5814:at the U.S. National Library of Medicine
5760:
5750:
5670:
5629:
5588:
5536:
5444:
5403:
5354:
5313:
5303:
5254:
5213:
5128:
5076:
5010:Journal of Bioenergetics and Biomembranes
4984:
4889:
4840:
4791:
4750:
4701:
4660:
4642:
4570:
4521:
4437:
4392:
4308:
4259:
4215:
4166:
4117:
4068:
4027:
4017:
3933:
3892:
3788:
3736:
3695:
3520:
3440:
3430:
3385:
3375:
3334:
3286:
3237:
3075:
3034:
2985:
2933:
2892:
2851:
1955:NADH dehydrogenase flavoprotein 3, 10kDa
1523:NADH dehydrogenase iron-sulfur protein 5
484:(PCD) during somatic embryo development.
6929:Mitochondrial membrane transport protein
2813:10.1146/annurev.biochem.75.103004.142539
2088:May or may not be present in any species
1126:NADH-ubiquinone oxidoreductase chain 4L
686:
476:Complex I may have a role in triggering
400:The reaction catalyzed by complex I is:
372:
360:
352:
29:
7533:
4504:Zharova TV, Vinogradov AD (July 1997).
3197:Principles of Biochemistry, 3rd Edition
2773:
1990:
1200:NADH-ubiquinone oxidoreductase chain 6
1156:NADH-ubiquinone oxidoreductase chain 5
1089:NADH-ubiquinone oxidoreductase chain 4
1059:NADH-ubiquinone oxidoreductase chain 3
1022:NADH-ubiquinone oxidoreductase chain 2
992:NADH-ubiquinone oxidoreductase chain 1
506:) downstream in the respiratory chain.
7568:. All relevant terms must be followed.
6688:Cholesterol side-chain cleavage enzyme
6367:: Oxidoreduction-driven transporters (
4915:Nature Reviews. Molecular Cell Biology
3154:Nature Reviews. Molecular Cell Biology
2779:
2777:
387:mitochondrial electron transport chain
149:
91:
5558:
5556:
4774:Galkin A, Moncada S (December 2007).
4098:The Journal of Clinical Investigation
3147:
3145:
3143:
3141:
3139:
2968:Batista AP, Pereira MM (March 2011).
2784:Berg J, Tymoczko J, Stryer L (2006).
2174:binding site of complex I as well as
385:Complex I is the first enzyme of the
7:
2337:(or semireduced flavin) to oxygen (O
6602:Coenzyme Q â cytochrome c reductase
5918:Methylenetetrahydrofolate reductase
5343:The Journal of Biological Chemistry
4833:10.1016/j.freeradbiomed.2018.06.040
4821:Free Radical Biology & Medicine
4780:The Journal of Biological Chemistry
3684:The Journal of Biological Chemistry
3419:The Journal of Biological Chemistry
3364:The Journal of Biological Chemistry
2439:), and some very young lineages of
2363:Leber's Hereditary Optic Neuropathy
1331:Acyl carrier protein, mitochondrial
391:Coenzyme Q â cytochrome c reductase
345:), Parkinson's disease and others.
6788:Oxoglutarate dehydrogenase complex
6723:Glycerol-3-phosphate dehydrogenase
6206:Complex II/Succinate dehydrogenase
6102:Pyruvate dehydrogenase phosphatase
5569:Antioxidants & Redox Signaling
5337:Galkin A, Brandt U (August 2005).
4196:American Journal of Human Genetics
2310:electron-transferring flavoprotein
2306:glycerol-3-phosphate dehydrogenase
25:
7554:, this article uses content from
6733:Carnitine palmitoyltransferase II
5631:10.1001/archgenpsychiatry.2010.22
3064:General Physiology and Biophysics
3058:Chomova M, Racay P (March 2010).
2085:Found in all species except fungi
7536:
6857:Carbamoyl phosphate synthetase I
6497:Long-chain-fatty-acidâCoA ligase
6492:Carnitine palmitoyltransferase I
5446:10.1111/j.1471-4159.2010.06631.x
5278:Kussmaul L, Hirst J (May 2006).
2091:Found in fungal species such as
1715:NADH dehydrogenase 1 subunit C2
688:Conserved subunits of Complex I
6718:Glutamate aspartate transporter
6230:Complex IV/Cytochrome c oxidase
5729:Sabater, B (19 November 2021).
2272:, ischemia or when the tissue
682:electron paramagnetic resonance
591:electron paramagnetic resonance
497:Na transporting Mrp superfamily
487:Complex I is not homologous to
18:NADH dehydrogenase (ubiquinone)
6835:Pyruvate dehydrogenase complex
6793:Succinyl coenzyme A synthetase
6077:Pyruvate dehydrogenase complex
5903:Dihydrolipoamide dehydrogenase
5618:Archives of General Psychiatry
2099:Recent research has described
568:Proton translocation mechanism
55:NADH:ubiquinone oxidoreductase
1:
6098:Pyruvate dehydrogenase kinase
4596:Current Pharmaceutical Design
4523:10.1016/S0005-2728(97)00029-7
4424:. 301 ( Pt 1) (Pt 1): 161â7.
4385:10.1016/j.febslet.2010.01.004
4310:10.1016/s0005-2728(98)00097-8
3763:, Hinchliffe P (March 2006).
3470:Journal of Structural Biology
3239:10.1016/s0005-2728(98)00027-9
2935:10.1016/s0014-5793(99)00575-x
2801:Annual Review of Biochemistry
2196:Complex I is also blocked by
6639:Dihydroorotate dehydrogenase
6201:Complex I/NADH dehydrogenase
5812:Electron+Transport+Complex+I
5807:Complex I news facebook page
5672:10.1016/j.bbadis.2010.02.001
5529:10.1161/STROKEAHA.117.019687
4743:10.1016/j.bbabio.2015.12.013
4694:10.1016/j.bbabio.2014.02.018
4346:10.1016/0378-8741(82)90002-2
4334:Journal of Ethnopharmacology
4070:10.1016/j.bbabio.2011.06.015
3959:Journal of Molecular Biology
3935:10.1016/j.bbabio.2004.06.001
3894:10.1016/j.bbabio.2010.05.009
3522:10.1016/j.bbabio.2011.04.006
3336:10.1016/j.bbabio.2012.02.015
2987:10.1016/j.bbabio.2010.12.008
2894:10.1016/j.bbabio.2006.10.001
2853:10.1016/0014-5793(84)80338-5
2321:concentrations are high and
2314:dihydroorotate dehydrogenase
2198:adenosine diphosphate ribose
6693:Steroid 11-beta-hydroxylase
5396:10.1016/j.neuro.2010.04.006
555:Electron transfer mechanism
431:difference used to produce
7613:
7597:Integral membrane proteins
6867:N-Acetylglutamate synthase
6862:Ornithine transcarbamylase
6666:Glycerol phosphate shuttle
6532:monoamine neurotransmitter
6021:Oxoglutarate dehydrogenase
4959:Murphy MP (January 2009).
4644:10.1007/s11357-023-01059-y
4608:10.2174/138161211796904795
4261:10.1016/j.cmet.2012.07.015
4208:10.1016/j.ajhg.2009.04.020
4051:Cardol P (November 2011).
3738:10.1016/j.cmet.2012.07.015
2235:Active/inactive transition
719:family with Human protein
7414:MichaelisâMenten kinetics
7221:
6919:
6895:
6592:oxidative phosphorylation
6415:Inorganic pyrophosphatase
6186:oxidative phosphorylation
5883:Butyryl CoA dehydrogenase
5878:Apoptosis-inducing factor
5863:
5708:10.1016/j.tox.2010.01.017
5482:10.1134/S0006297919110154
5433:Journal of Neurochemistry
5057:Journal of Neurochemistry
3971:10.1016/j.jmb.2005.02.067
3820:Efremov RG, Baradaran R,
3503:Brandt U (October 2011).
3482:10.1016/j.jsb.2007.01.009
3114:10.1007/s00425-009-1028-x
2834:Wikström M (April 1984).
2094:Schizosaccharomyces pombe
1987:Assembly factor proteins
1986:
1218:
723:
629:Composition and structure
564:the proton motive force.
429:electrochemical potential
281:
59:Type I NADH dehydrogenase
7306:Diffusion-limited enzyme
6783:Isocitrate dehydrogenase
6661:Malate-aspartate shuttle
6181:electron transport chain
6141:Methylmalonyl-CoA mutase
6016:Isocitrate dehydrogenase
5816:Medical Subject Headings
5470:Biochemistry. Biokhimiia
5121:10.1177/0271678X17730242
4882:10.1177/0271678X18770331
4159:10.1038/sj.emboj.7601748
3645:Voet JG, Voet D (2004).
3464:Baranova EA, Morgan DJ,
2455:for so long is unknown.
2288:. Complex I can produce
2280:Production of superoxide
1219:Core accessory subunits
448:Paracoccus denitrificans
6830:Glutamate dehydrogenase
6617:Succinate dehydrogenase
6122:Glutamate dehydrogenase
6033:Succinate dehydrogenase
6026:Succinyl CoA synthetase
5913:Methemoglobin reductase
5898:Cytokinin dehydrogenase
5893:Cytochrome b5 reductase
5305:10.1073/pnas.0510977103
5155:The Biochemical Journal
5022:10.1023/A:1022420007908
4965:The Biochemical Journal
4422:The Biochemical Journal
4019:10.1073/pnas.0908050107
3790:10.1126/science.1123809
3565:10.1126/science.1259859
3432:10.1074/jbc.m109.032144
3377:10.1074/jbc.M111.252502
2302:succinate dehydrogenase
2286:reactive oxygen species
427:, helping to build the
63:mitochondrial complex I
7226:mitochondrial diseases
6825:Aspartate transaminase
6482:fatty acid degradation
6410:Cytochrome b6f complex
6165:Aspartate transaminase
5873:Acyl CoA dehydrogenase
5356:10.1074/jbc.M504709200
4793:10.1074/jbc.M707543200
4475:10.1124/mol.108.048546
4463:Molecular Pharmacology
3697:10.1074/jbc.M607135200
3220:Ohnishi T (May 1998).
3199:. Wiley. p. 608.
2351:mitochondrial diseases
2208:The antidiabetic drug
2107:, and not of complex I
382:
370:
358:
40:
7399:EadieâHofstee diagram
7332:Allosteric regulation
6939:Mitochondrial carrier
6815:anaplerotic reactions
6651:mitochondrial shuttle
6629:pyrimidine metabolism
5943:Thioredoxin reductase
5868:Acetolactate synthase
5752:10.3390/ijms222212505
5581:10.1089/ars.2018.7693
3077:10.4149/gpb_2010_01_3
2361:) can also result in
2202:competitive inhibitor
709:Protein description (
521:flavin mononucleotide
482:programmed cell death
376:
364:
356:
108:Respiratory complex I
95:Respiratory complex I
65:) is the first large
44:Respiratory complex I
33:
7582:Cellular respiration
7564:, but not under the
7409:LineweaverâBurk plot
6946:Translocator protein
6803:Malate dehydrogenase
6698:Aldosterone synthase
6160:Pyruvate carboxylase
6048:Malate dehydrogenase
948:NDUFS1 / 75kD / NUAM
912:NDUFV1 / 51kD / NUBM
876:NDUFS2 / 49kD / NUCM
840:NDUFS3 / 30kD / NUGM
804:NDUFV2 / 24kD / NUHM
768:NDUFS8 / TYKY / NUIM
732:NDUFS7 / PSST / NUKM
680:A recent study used
650:mitochondrial genome
646:iron-sulfur clusters
597:formation step from
577:groups of Complexes
458:Rhodothermus marinus
395:cytochrome c oxidase
335:ischemia/reperfusion
6558:Intermembrane space
6310:Alternative oxidase
6114:α-ketoglutaric acid
5296:2006PNAS..103.7607K
5206:10.1038/pr.2017.277
4010:2010PNAS..107.1930R
3846:10.1038/nature09066
3838:2010Natur.465..441E
3781:2006Sci...311.1430S
3616:10.1038/nature03692
3608:2005Natur.435.1197H
2367:Parkinson's disease
2103:to be a subunit of
689:
495:), a member of the
404:NADH + H + CoQ + 4H
393:(complex III), and
7368:Enzyme superfamily
7301:Enzyme promiscuity
6913:Other/to be sorted
6878:alcohol metabolism
6738:Uncoupling protein
6612:NADH dehydrogenase
5923:NADH dehydrogenase
5802:Complex I homepage
5194:Pediatric Research
5167:10.1042/BJ20071162
4977:10.1042/BJ20081386
4563:10.1042/CS20110386
3602:(7046): 1197â202.
687:
383:
371:
359:
343:cardiac infarction
71:respiratory chains
41:
7524:
7523:
7233:
7232:
7217:
7216:
6960:Mitochondrial DNA
6954:
6953:
6908:
6907:
6763:citric acid cycle
6746:
6745:
6552:
6551:
6544:Monoamine oxidase
6428:
6427:
6327:
6326:
6323:
6322:
6173:
6172:
5989:Citric acid cycle
5951:
5950:
5938:Sarcosine oxidase
5933:Nitrate reductase
5476:(11): 1411â1423.
5256:10.1021/bi702243b
5115:(12): 3649â3658.
5069:10.1111/jnc.14654
4430:10.1042/bj3010161
3279:10.1021/bi201644x
3206:978-0-470-23396-2
3027:10.1021/bi7009822
2294:hydrogen peroxide
2245:activation energy
2079:
2078:
669:(PhaA and PhaD).
625:of the residues.
515:Overall mechanism
330:
329:
326:
325:
229:metabolic pathway
148:
147:
16:(Redirected from
7604:
7541:
7540:
7532:
7404:HanesâWoolf plot
7347:Enzyme activator
7342:Enzyme inhibitor
7316:Enzyme catalysis
7260:
7253:
7246:
7237:
7097:
7072:
7042:
7022:
6972:
6965:
6917:
6880:
6850:
6818:
6773:Citrate synthase
6766:
6757:
6711:
6681:
6654:
6632:
6595:
6586:
6566:Adenylate kinase
6537:
6513:
6485:
6476:
6455:
6448:
6441:
6432:
6354:
6347:
6340:
6331:
6191:
6152:oxaloacetic acid
6064:
6006:Citrate synthase
5978:
5971:
5964:
5955:
5846:
5839:
5832:
5823:
5775:
5774:
5764:
5754:
5726:
5720:
5719:
5691:
5685:
5684:
5674:
5650:
5644:
5643:
5633:
5609:
5603:
5602:
5592:
5560:
5551:
5550:
5540:
5523:(5): 1223â1231.
5508:
5502:
5501:
5465:
5459:
5458:
5448:
5424:
5418:
5417:
5407:
5375:
5369:
5368:
5358:
5349:(34): 30129â35.
5334:
5328:
5327:
5317:
5307:
5275:
5269:
5268:
5258:
5234:
5228:
5227:
5217:
5185:
5179:
5178:
5149:
5143:
5142:
5132:
5100:
5091:
5090:
5080:
5048:
5042:
5041:
5005:
4999:
4998:
4988:
4956:
4947:
4946:
4910:
4904:
4903:
4893:
4876:(9): 1790â1802.
4861:
4855:
4854:
4844:
4812:
4806:
4805:
4795:
4786:(52): 37448â53.
4771:
4765:
4764:
4754:
4722:
4716:
4715:
4705:
4673:
4667:
4666:
4664:
4646:
4626:
4620:
4619:
4591:
4585:
4584:
4574:
4551:Clinical Science
4542:
4536:
4535:
4525:
4501:
4495:
4494:
4458:
4452:
4451:
4441:
4413:
4407:
4406:
4396:
4364:
4358:
4357:
4329:
4323:
4322:
4312:
4288:
4282:
4281:
4263:
4239:
4230:
4229:
4219:
4187:
4181:
4180:
4170:
4147:The EMBO Journal
4138:
4132:
4131:
4121:
4110:10.1172/JCI26020
4089:
4083:
4082:
4072:
4048:
4042:
4041:
4031:
4021:
3989:
3983:
3982:
3954:
3948:
3947:
3937:
3913:
3907:
3906:
3896:
3872:
3866:
3865:
3817:
3811:
3810:
3792:
3775:(5766): 1430â6.
3757:
3751:
3750:
3740:
3716:
3710:
3709:
3699:
3675:
3669:
3668:
3652:
3642:
3636:
3635:
3591:
3585:
3584:
3550:
3541:
3535:
3534:
3524:
3500:
3494:
3493:
3461:
3455:
3454:
3444:
3434:
3425:(43): 29773â83.
3413:(October 2009).
3406:
3400:
3399:
3389:
3379:
3370:(31): 27103â10.
3355:
3349:
3348:
3338:
3317:(October 2012).
3310:
3301:
3300:
3290:
3258:
3252:
3251:
3241:
3217:
3211:
3210:
3192:
3186:
3185:
3149:
3134:
3133:
3096:
3090:
3089:
3079:
3055:
3049:
3048:
3038:
3006:
3000:
2999:
2989:
2965:
2956:
2955:
2937:
2913:
2907:
2906:
2896:
2872:
2866:
2865:
2855:
2831:
2825:
2824:
2796:
2790:
2789:
2781:
2253:N-Ethylmaleimide
690:
644:(FMN) and eight
642:prosthetic group
463:P. denitrificans
438:Escherichia coli
283:
150:
92:
21:
7612:
7611:
7607:
7606:
7605:
7603:
7602:
7601:
7572:
7571:
7547:
7535:
7527:
7525:
7520:
7432:Oxidoreductases
7418:
7394:Enzyme kinetics
7382:
7378:List of enzymes
7351:
7320:
7291:Catalytic triad
7269:
7264:
7234:
7229:
7213:
7093:
7088:
7068:
7063:
7038:
7033:
7018:
7013:
6968:
6950:
6904:
6891:
6876:
6871:
6844:
6839:
6812:
6807:
6760:
6742:
6707:
6702:
6678:steroidogenesis
6675:
6670:
6648:
6643:
6626:
6621:
6589:
6575:
6571:Creatine kinase
6548:
6534:
6529:
6524:
6506:
6501:
6479:
6465:
6459:
6429:
6424:
6398:
6389:ETC Complex III
6372:
6358:
6328:
6319:
6298:
6240:
6214:
6184:
6179:
6169:
6145:
6126:
6107:
6053:
5994:
5982:
5952:
5947:
5859:
5850:
5783:
5778:
5728:
5727:
5723:
5693:
5692:
5688:
5652:
5651:
5647:
5611:
5610:
5606:
5562:
5561:
5554:
5510:
5509:
5505:
5467:
5466:
5462:
5426:
5425:
5421:
5384:Neurotoxicology
5377:
5376:
5372:
5336:
5335:
5331:
5290:(20): 7607â12.
5277:
5276:
5272:
5249:(12): 3964â71.
5236:
5235:
5231:
5187:
5186:
5182:
5151:
5150:
5146:
5102:
5101:
5094:
5050:
5049:
5045:
5007:
5006:
5002:
4958:
4957:
4950:
4912:
4911:
4907:
4863:
4862:
4858:
4814:
4813:
4809:
4773:
4772:
4768:
4724:
4723:
4719:
4675:
4674:
4670:
4628:
4627:
4623:
4602:(20): 2100â12.
4593:
4592:
4588:
4544:
4543:
4539:
4503:
4502:
4498:
4460:
4459:
4455:
4415:
4414:
4410:
4366:
4365:
4361:
4331:
4330:
4326:
4290:
4289:
4285:
4248:Cell Metabolism
4241:
4240:
4233:
4189:
4188:
4184:
4153:(13): 3227â37.
4140:
4139:
4135:
4104:(10): 2784â92.
4091:
4090:
4086:
4050:
4049:
4045:
3991:
3990:
3986:
3956:
3955:
3951:
3915:
3914:
3910:
3887:(12): 1883â90.
3874:
3873:
3869:
3832:(7297): 441â5.
3819:
3818:
3814:
3759:
3758:
3754:
3725:Cell Metabolism
3718:
3717:
3713:
3690:(43): 32724â7.
3677:
3676:
3672:
3665:
3644:
3643:
3639:
3593:
3592:
3588:
3548:
3543:
3542:
3538:
3502:
3501:
3497:
3463:
3462:
3458:
3409:Berrisford JM,
3408:
3407:
3403:
3357:
3356:
3352:
3329:(10): 1785â95.
3312:
3311:
3304:
3260:
3259:
3255:
3219:
3218:
3214:
3207:
3194:
3193:
3189:
3166:10.1038/nrm3997
3151:
3150:
3137:
3098:
3097:
3093:
3057:
3056:
3052:
3021:(38): 10971â8.
3008:
3007:
3003:
2967:
2966:
2959:
2915:
2914:
2910:
2887:(12): 1575â81.
2874:
2873:
2869:
2833:
2832:
2828:
2798:
2797:
2793:
2783:
2782:
2775:
2771:
2461:
2423:under the name
2417:
2415:In chloroplasts
2402:
2347:
2340:
2336:
2282:
2237:
2200:â a reversible
2134:
2126:Asimina triloba
2114:
1755:NDUFA7 / B14.5A
1707:NDUFC2 / B14.5B
1467:NDUFA11 / B14.7
1251:NDUFA12 / B17.2
631:
624:
570:
557:
550:
526:
517:
512:
415:
411:
407:
380:
368:
351:
114:OPM superfamily
67:protein complex
53:(also known as
38:
28:
23:
22:
15:
12:
11:
5:
7610:
7608:
7600:
7599:
7594:
7589:
7584:
7574:
7573:
7546:
7545:
7522:
7521:
7519:
7518:
7505:
7492:
7479:
7466:
7453:
7440:
7426:
7424:
7420:
7419:
7417:
7416:
7411:
7406:
7401:
7396:
7390:
7388:
7384:
7383:
7381:
7380:
7375:
7370:
7365:
7359:
7357:
7356:Classification
7353:
7352:
7350:
7349:
7344:
7339:
7334:
7328:
7326:
7322:
7321:
7319:
7318:
7313:
7308:
7303:
7298:
7293:
7288:
7283:
7277:
7275:
7271:
7270:
7265:
7263:
7262:
7255:
7248:
7240:
7231:
7230:
7222:
7219:
7218:
7215:
7214:
7212:
7211:
7206:
7201:
7196:
7191:
7186:
7181:
7176:
7171:
7166:
7161:
7156:
7151:
7146:
7141:
7136:
7131:
7126:
7121:
7116:
7111:
7106:
7100:
7098:
7090:
7089:
7087:
7086:
7081:
7075:
7073:
7065:
7064:
7062:
7061:
7056:
7051:
7045:
7043:
7035:
7034:
7032:
7031:
7025:
7023:
7015:
7014:
7012:
7011:
7006:
7001:
6996:
6991:
6986:
6981:
6975:
6973:
6962:
6956:
6955:
6952:
6951:
6949:
6948:
6943:
6942:
6941:
6936:
6926:
6920:
6914:
6910:
6909:
6906:
6905:
6903:
6902:
6896:
6893:
6892:
6890:
6889:
6883:
6881:
6873:
6872:
6870:
6869:
6864:
6859:
6853:
6851:
6841:
6840:
6838:
6837:
6832:
6827:
6821:
6819:
6809:
6808:
6806:
6805:
6800:
6795:
6790:
6785:
6780:
6775:
6769:
6767:
6754:
6748:
6747:
6744:
6743:
6741:
6740:
6735:
6730:
6725:
6720:
6714:
6712:
6704:
6703:
6701:
6700:
6695:
6690:
6684:
6682:
6672:
6671:
6669:
6668:
6663:
6657:
6655:
6645:
6644:
6642:
6641:
6635:
6633:
6623:
6622:
6620:
6619:
6614:
6609:
6604:
6598:
6596:
6583:
6581:Inner membrane
6577:
6576:
6574:
6573:
6568:
6562:
6560:
6554:
6553:
6550:
6549:
6547:
6546:
6540:
6538:
6526:
6525:
6523:
6522:
6516:
6514:
6503:
6502:
6500:
6499:
6494:
6488:
6486:
6473:
6471:Outer membrane
6467:
6466:
6460:
6458:
6457:
6450:
6443:
6435:
6426:
6425:
6423:
6422:
6417:
6412:
6406:
6404:
6400:
6399:
6397:
6396:
6394:ETC Complex IV
6391:
6386:
6380:
6378:
6374:
6373:
6359:
6357:
6356:
6349:
6342:
6334:
6325:
6324:
6321:
6320:
6318:
6317:
6312:
6306:
6304:
6300:
6299:
6297:
6296:
6291:
6286:
6281:
6276:
6271:
6266:
6261:
6256:
6251:
6246:
6238:
6233:
6232:
6227:
6222:
6217:
6212:
6208:
6203:
6197:
6195:
6188:
6175:
6174:
6171:
6170:
6168:
6167:
6162:
6156:
6154:
6147:
6146:
6144:
6143:
6137:
6135:
6128:
6127:
6125:
6124:
6118:
6116:
6109:
6108:
6106:
6105:
6096:(regulated by
6093:
6092:
6073:
6071:
6061:
6055:
6054:
6052:
6051:
6045:
6040:
6029:
6028:
6023:
6018:
6013:
6008:
6002:
6000:
5996:
5995:
5983:
5981:
5980:
5973:
5966:
5958:
5949:
5948:
5946:
5945:
5940:
5935:
5930:
5925:
5920:
5915:
5910:
5905:
5900:
5895:
5890:
5885:
5880:
5875:
5870:
5864:
5861:
5860:
5851:
5849:
5848:
5841:
5834:
5826:
5820:
5819:
5809:
5804:
5799:
5789:
5782:
5781:External links
5779:
5777:
5776:
5721:
5702:(2â3): 77â84.
5686:
5645:
5604:
5575:(9): 608â622.
5552:
5503:
5460:
5419:
5370:
5329:
5270:
5229:
5200:(2): 491â497.
5180:
5144:
5092:
5063:(6): 731â745.
5043:
5000:
4948:
4927:10.1038/nrm762
4905:
4856:
4807:
4766:
4717:
4688:(7): 1083â92.
4668:
4621:
4586:
4537:
4496:
4453:
4408:
4359:
4324:
4283:
4254:(3): 378â386.
4231:
4182:
4133:
4084:
4063:(11): 1390â7.
4043:
3984:
3949:
3908:
3867:
3812:
3752:
3711:
3670:
3663:
3637:
3586:
3559:(6217): 44â9.
3536:
3515:(10): 1364â9.
3495:
3456:
3401:
3350:
3302:
3253:
3232:(2): 186â206.
3212:
3205:
3187:
3135:
3091:
3050:
3001:
2957:
2908:
2867:
2826:
2791:
2772:
2770:
2767:
2766:
2765:
2764:
2763:
2757:
2751:
2745:
2739:
2733:
2727:
2718:
2717:
2716:
2710:
2704:
2695:
2694:
2693:
2687:
2681:
2675:
2669:
2663:
2657:
2651:
2642:
2641:
2640:
2634:
2625:
2624:
2623:
2617:
2611:
2605:
2599:
2593:
2587:
2584:
2581:
2578:
2572:
2563:
2562:
2561:
2558:
2555:
2552:
2546:
2543:
2537:
2531:
2528:
2522:
2516:
2510:
2507:
2501:
2495:
2492:
2489:
2483:
2480:
2474:
2460:
2457:
2416:
2413:
2400:
2355:Leigh syndrome
2346:
2343:
2338:
2334:
2281:
2278:
2236:
2233:
2217:hepatotoxicity
2132:
2113:
2110:
2109:
2108:
2097:
2089:
2086:
2077:
2076:
2068:
2065:
2062:
2059:
2055:
2054:
2046:
2043:
2040:
2037:
2033:
2032:
2024:
2021:
2018:
2015:
2011:
2010:
2002:
1999:
1996:
1993:
1989:
1988:
1984:
1983:
1975:
1972:
1967:
1964:
1960:
1959:
1956:
1953:
1948:
1945:
1941:
1940:
1932:
1929:
1924:
1921:
1917:
1916:
1908:
1905:
1900:
1899:NDUFA10 / 42kD
1897:
1893:
1892:
1884:
1881:
1876:
1873:
1869:
1868:
1860:
1857:
1852:
1849:
1845:
1844:
1836:
1833:
1828:
1825:
1821:
1820:
1812:
1809:
1804:
1801:
1797:
1796:
1788:
1785:
1780:
1777:
1773:
1772:
1764:
1761:
1756:
1753:
1749:
1748:
1740:
1737:
1732:
1729:
1725:
1724:
1716:
1713:
1708:
1705:
1701:
1700:
1692:
1689:
1684:
1681:
1677:
1676:
1668:
1665:
1660:
1659:NDUFB10 / PDSW
1657:
1653:
1652:
1644:
1641:
1636:
1633:
1629:
1628:
1620:
1617:
1612:
1609:
1605:
1604:
1596:
1593:
1588:
1585:
1581:
1580:
1572:
1569:
1564:
1561:
1557:
1556:
1548:
1545:
1540:
1537:
1533:
1532:
1524:
1521:
1516:
1513:
1509:
1508:
1500:
1497:
1492:
1491:NDUFB11 / ESSS
1489:
1485:
1484:
1476:
1473:
1468:
1465:
1461:
1460:
1452:
1449:
1444:
1441:
1437:
1436:
1428:
1425:
1420:
1417:
1413:
1412:
1404:
1401:
1396:
1393:
1389:
1388:
1380:
1377:
1372:
1369:
1365:
1364:
1356:
1353:
1348:
1345:
1341:
1340:
1332:
1329:
1324:
1323:NDUFAB1 / ACPM
1321:
1317:
1316:
1308:
1305:
1300:
1299:NDUFA9 / 39kDa
1297:
1293:
1292:
1284:
1281:
1276:
1273:
1269:
1268:
1260:
1257:
1252:
1249:
1245:
1244:
1236:
1233:
1228:
1225:
1221:
1220:
1216:
1215:
1207:
1198:
1193:
1190:
1186:
1185:
1163:
1154:
1149:
1146:
1142:
1141:
1133:
1124:
1119:
1116:
1112:
1111:
1096:
1087:
1082:
1079:
1075:
1074:
1066:
1057:
1052:
1049:
1045:
1044:
1029:
1020:
1015:
1012:
1008:
1007:
999:
990:
985:
982:
978:
977:
969:
954:
949:
946:
942:
941:
933:
918:
913:
910:
906:
905:
897:
882:
877:
874:
870:
869:
861:
846:
841:
838:
834:
833:
825:
810:
805:
802:
798:
797:
789:
774:
769:
766:
762:
761:
753:
738:
733:
730:
726:
725:
724:Core Subunits
721:
720:
714:
707:
706:Human protein
704:
694:
667:TC# 2.A.63.1.1
630:
627:
622:
612:The resulting
569:
566:
556:
553:
548:
524:
516:
513:
511:
508:
417:
416:
413:
409:
405:
378:
366:
350:
347:
328:
327:
324:
323:
318:
312:
311:
306:
300:
299:
294:
288:
287:
279:
278:
269:
263:
262:
251:
244:
243:
238:
232:
231:
226:
220:
219:
214:
208:
207:
202:
196:
195:
190:
184:
183:
178:
172:
171:
167:
166:
161:
155:
154:
146:
145:
140:
134:
133:
128:
122:
121:
116:
110:
109:
106:
102:
101:
97:
96:
36:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
7609:
7598:
7595:
7593:
7590:
7588:
7585:
7583:
7580:
7579:
7577:
7570:
7569:
7567:
7563:
7558:
7555:
7553:
7544:
7539:
7534:
7530:
7516:
7512:
7511:
7506:
7503:
7499:
7498:
7493:
7490:
7486:
7485:
7480:
7477:
7473:
7472:
7467:
7464:
7460:
7459:
7454:
7451:
7447:
7446:
7441:
7438:
7434:
7433:
7428:
7427:
7425:
7421:
7415:
7412:
7410:
7407:
7405:
7402:
7400:
7397:
7395:
7392:
7391:
7389:
7385:
7379:
7376:
7374:
7373:Enzyme family
7371:
7369:
7366:
7364:
7361:
7360:
7358:
7354:
7348:
7345:
7343:
7340:
7338:
7337:Cooperativity
7335:
7333:
7330:
7329:
7327:
7323:
7317:
7314:
7312:
7309:
7307:
7304:
7302:
7299:
7297:
7296:Oxyanion hole
7294:
7292:
7289:
7287:
7284:
7282:
7279:
7278:
7276:
7272:
7268:
7261:
7256:
7254:
7249:
7247:
7242:
7241:
7238:
7228:
7227:
7220:
7210:
7207:
7205:
7202:
7200:
7197:
7195:
7192:
7190:
7187:
7185:
7182:
7180:
7177:
7175:
7172:
7170:
7167:
7165:
7162:
7160:
7157:
7155:
7152:
7150:
7147:
7145:
7142:
7140:
7137:
7135:
7132:
7130:
7127:
7125:
7122:
7120:
7117:
7115:
7112:
7110:
7107:
7105:
7102:
7101:
7099:
7096:
7091:
7085:
7082:
7080:
7077:
7076:
7074:
7071:
7066:
7060:
7057:
7055:
7052:
7050:
7047:
7046:
7044:
7041:
7036:
7030:
7027:
7026:
7024:
7021:
7016:
7010:
7007:
7005:
7002:
7000:
6997:
6995:
6992:
6990:
6987:
6985:
6982:
6980:
6977:
6976:
6974:
6971:
6966:
6963:
6961:
6957:
6947:
6944:
6940:
6937:
6935:
6932:
6931:
6930:
6927:
6925:
6922:
6921:
6918:
6915:
6911:
6901:
6898:
6897:
6894:
6888:
6885:
6884:
6882:
6879:
6874:
6868:
6865:
6863:
6860:
6858:
6855:
6854:
6852:
6849:
6848:
6842:
6836:
6833:
6831:
6828:
6826:
6823:
6822:
6820:
6817:
6816:
6810:
6804:
6801:
6799:
6796:
6794:
6791:
6789:
6786:
6784:
6781:
6779:
6776:
6774:
6771:
6770:
6768:
6765:
6764:
6758:
6755:
6753:
6749:
6739:
6736:
6734:
6731:
6729:
6726:
6724:
6721:
6719:
6716:
6715:
6713:
6710:
6705:
6699:
6696:
6694:
6691:
6689:
6686:
6685:
6683:
6680:
6679:
6673:
6667:
6664:
6662:
6659:
6658:
6656:
6653:
6652:
6646:
6640:
6637:
6636:
6634:
6631:
6630:
6624:
6618:
6615:
6613:
6610:
6608:
6605:
6603:
6600:
6599:
6597:
6594:
6593:
6587:
6584:
6582:
6578:
6572:
6569:
6567:
6564:
6563:
6561:
6559:
6555:
6545:
6542:
6541:
6539:
6536:
6533:
6527:
6521:
6518:
6517:
6515:
6512:
6510:
6504:
6498:
6495:
6493:
6490:
6489:
6487:
6484:
6483:
6477:
6474:
6472:
6468:
6463:
6462:Mitochondrial
6456:
6451:
6449:
6444:
6442:
6437:
6436:
6433:
6421:
6418:
6416:
6413:
6411:
6408:
6407:
6405:
6401:
6395:
6392:
6390:
6387:
6385:
6384:ETC Complex I
6382:
6381:
6379:
6375:
6370:
6366:
6362:
6355:
6350:
6348:
6343:
6341:
6336:
6335:
6332:
6316:
6313:
6311:
6308:
6307:
6305:
6301:
6295:
6292:
6290:
6287:
6285:
6282:
6280:
6277:
6275:
6272:
6270:
6267:
6265:
6262:
6260:
6257:
6255:
6252:
6250:
6247:
6245:
6241:
6235:
6234:
6231:
6228:
6226:
6223:
6221:
6218:
6216:
6209:
6207:
6204:
6202:
6199:
6198:
6196:
6192:
6189:
6187:
6182:
6178:Mitochondrial
6176:
6166:
6163:
6161:
6158:
6157:
6155:
6153:
6148:
6142:
6139:
6138:
6136:
6134:
6129:
6123:
6120:
6119:
6117:
6115:
6110:
6103:
6099:
6095:
6094:
6090:
6086:
6082:
6078:
6075:
6074:
6072:
6070:
6065:
6062:
6060:
6056:
6049:
6046:
6044:
6041:
6038:
6034:
6031:
6030:
6027:
6024:
6022:
6019:
6017:
6014:
6012:
6009:
6007:
6004:
6003:
6001:
5997:
5993:
5990:
5986:
5979:
5974:
5972:
5967:
5965:
5960:
5959:
5956:
5944:
5941:
5939:
5936:
5934:
5931:
5929:
5928:NADPH oxidase
5926:
5924:
5921:
5919:
5916:
5914:
5911:
5909:
5906:
5904:
5901:
5899:
5896:
5894:
5891:
5889:
5886:
5884:
5881:
5879:
5876:
5874:
5871:
5869:
5866:
5865:
5862:
5858:
5857:flavoproteins
5854:
5847:
5842:
5840:
5835:
5833:
5828:
5827:
5824:
5817:
5813:
5810:
5808:
5805:
5803:
5800:
5797:
5793:
5790:
5788:
5785:
5784:
5780:
5772:
5768:
5763:
5758:
5753:
5748:
5745:(22): 12505.
5744:
5740:
5736:
5734:
5725:
5722:
5717:
5713:
5709:
5705:
5701:
5697:
5690:
5687:
5682:
5678:
5673:
5668:
5665:(5): 443â53.
5664:
5660:
5656:
5649:
5646:
5641:
5637:
5632:
5627:
5623:
5619:
5615:
5608:
5605:
5600:
5596:
5591:
5586:
5582:
5578:
5574:
5570:
5566:
5559:
5557:
5553:
5548:
5544:
5539:
5534:
5530:
5526:
5522:
5518:
5514:
5507:
5504:
5499:
5495:
5491:
5487:
5483:
5479:
5475:
5471:
5464:
5461:
5456:
5452:
5447:
5442:
5439:(3): 674â82.
5438:
5434:
5430:
5423:
5420:
5415:
5411:
5406:
5401:
5397:
5393:
5390:(4): 367â72.
5389:
5385:
5381:
5374:
5371:
5366:
5362:
5357:
5352:
5348:
5344:
5340:
5333:
5330:
5325:
5321:
5316:
5311:
5306:
5301:
5297:
5293:
5289:
5285:
5281:
5274:
5271:
5266:
5262:
5257:
5252:
5248:
5244:
5240:
5233:
5230:
5225:
5221:
5216:
5211:
5207:
5203:
5199:
5195:
5191:
5184:
5181:
5176:
5172:
5168:
5164:
5160:
5156:
5148:
5145:
5140:
5136:
5131:
5126:
5122:
5118:
5114:
5110:
5106:
5099:
5097:
5093:
5088:
5084:
5079:
5074:
5070:
5066:
5062:
5058:
5054:
5047:
5044:
5039:
5035:
5031:
5027:
5023:
5019:
5015:
5011:
5004:
5001:
4996:
4992:
4987:
4982:
4978:
4974:
4970:
4966:
4962:
4955:
4953:
4949:
4944:
4940:
4936:
4932:
4928:
4924:
4921:(3): 214â20.
4920:
4916:
4909:
4906:
4901:
4897:
4892:
4887:
4883:
4879:
4875:
4871:
4867:
4860:
4857:
4852:
4848:
4843:
4838:
4834:
4830:
4826:
4822:
4818:
4811:
4808:
4803:
4799:
4794:
4789:
4785:
4781:
4777:
4770:
4767:
4762:
4758:
4753:
4748:
4744:
4740:
4737:(7): 946â57.
4736:
4732:
4728:
4721:
4718:
4713:
4709:
4704:
4699:
4695:
4691:
4687:
4683:
4679:
4672:
4669:
4663:
4658:
4654:
4650:
4645:
4640:
4636:
4632:
4625:
4622:
4617:
4613:
4609:
4605:
4601:
4597:
4590:
4587:
4582:
4578:
4573:
4568:
4564:
4560:
4557:(6): 253â70.
4556:
4552:
4548:
4541:
4538:
4533:
4529:
4524:
4519:
4516:(3): 256â64.
4515:
4511:
4507:
4500:
4497:
4492:
4488:
4484:
4480:
4476:
4472:
4469:(4): 933â40.
4468:
4464:
4457:
4454:
4449:
4445:
4440:
4435:
4431:
4427:
4423:
4419:
4412:
4409:
4404:
4400:
4395:
4390:
4386:
4382:
4378:
4374:
4370:
4363:
4360:
4355:
4351:
4347:
4343:
4340:(2): 139â60.
4339:
4336:(in French).
4335:
4328:
4325:
4320:
4316:
4311:
4306:
4303:(3): 443â52.
4302:
4298:
4294:
4287:
4284:
4279:
4275:
4271:
4267:
4262:
4257:
4253:
4249:
4245:
4238:
4236:
4232:
4227:
4223:
4218:
4213:
4209:
4205:
4202:(6): 718â27.
4201:
4197:
4193:
4186:
4183:
4178:
4174:
4169:
4164:
4160:
4156:
4152:
4148:
4144:
4137:
4134:
4129:
4125:
4120:
4115:
4111:
4107:
4103:
4099:
4095:
4088:
4085:
4080:
4076:
4071:
4066:
4062:
4058:
4054:
4047:
4044:
4039:
4035:
4030:
4025:
4020:
4015:
4011:
4007:
4004:(5): 1930â5.
4003:
3999:
3995:
3988:
3985:
3980:
3976:
3972:
3968:
3965:(4): 857â70.
3964:
3960:
3953:
3950:
3945:
3941:
3936:
3931:
3928:(3): 212â24.
3927:
3923:
3919:
3912:
3909:
3904:
3900:
3895:
3890:
3886:
3882:
3878:
3871:
3868:
3863:
3859:
3855:
3851:
3847:
3843:
3839:
3835:
3831:
3827:
3823:
3816:
3813:
3808:
3804:
3800:
3796:
3791:
3786:
3782:
3778:
3774:
3770:
3766:
3762:
3756:
3753:
3748:
3744:
3739:
3734:
3731:(3): 378â86.
3730:
3726:
3722:
3715:
3712:
3707:
3703:
3698:
3693:
3689:
3685:
3681:
3674:
3671:
3666:
3664:0-471-19350-X
3660:
3656:
3651:
3650:
3641:
3638:
3633:
3629:
3625:
3621:
3617:
3613:
3609:
3605:
3601:
3597:
3590:
3587:
3582:
3578:
3574:
3570:
3566:
3562:
3558:
3554:
3547:
3540:
3537:
3532:
3528:
3523:
3518:
3514:
3510:
3506:
3499:
3496:
3491:
3487:
3483:
3479:
3476:(2): 238â42.
3475:
3471:
3467:
3460:
3457:
3452:
3448:
3443:
3438:
3433:
3428:
3424:
3420:
3416:
3412:
3405:
3402:
3397:
3393:
3388:
3383:
3378:
3373:
3369:
3365:
3361:
3354:
3351:
3346:
3342:
3337:
3332:
3328:
3324:
3320:
3316:
3309:
3307:
3303:
3298:
3294:
3289:
3284:
3280:
3276:
3273:(1): 149â58.
3272:
3268:
3264:
3257:
3254:
3249:
3245:
3240:
3235:
3231:
3227:
3223:
3216:
3213:
3208:
3202:
3198:
3191:
3188:
3183:
3179:
3175:
3171:
3167:
3163:
3160:(6): 375â88.
3159:
3155:
3148:
3146:
3144:
3142:
3140:
3136:
3131:
3127:
3123:
3119:
3115:
3111:
3108:(1): 93â107.
3107:
3103:
3095:
3092:
3087:
3083:
3078:
3073:
3069:
3065:
3061:
3054:
3051:
3046:
3042:
3037:
3032:
3028:
3024:
3020:
3016:
3012:
3005:
3002:
2997:
2993:
2988:
2983:
2980:(3): 286â92.
2979:
2975:
2971:
2964:
2962:
2958:
2953:
2949:
2945:
2941:
2936:
2931:
2928:(2): 157â61.
2927:
2923:
2919:
2912:
2909:
2904:
2900:
2895:
2890:
2886:
2882:
2878:
2871:
2868:
2863:
2859:
2854:
2849:
2845:
2841:
2837:
2830:
2827:
2822:
2818:
2814:
2810:
2806:
2802:
2795:
2792:
2787:
2780:
2778:
2774:
2768:
2761:
2758:
2755:
2752:
2749:
2746:
2743:
2740:
2737:
2734:
2731:
2728:
2725:
2722:
2721:
2719:
2714:
2711:
2708:
2705:
2702:
2699:
2698:
2696:
2691:
2688:
2685:
2682:
2679:
2676:
2673:
2670:
2667:
2664:
2661:
2658:
2655:
2652:
2649:
2646:
2645:
2643:
2638:
2635:
2632:
2629:
2628:
2626:
2621:
2618:
2615:
2612:
2609:
2606:
2603:
2600:
2597:
2594:
2591:
2588:
2585:
2582:
2579:
2576:
2573:
2570:
2567:
2566:
2564:
2559:
2556:
2553:
2550:
2547:
2544:
2541:
2538:
2535:
2532:
2529:
2526:
2523:
2520:
2517:
2514:
2511:
2508:
2505:
2502:
2499:
2496:
2493:
2490:
2487:
2484:
2481:
2478:
2475:
2472:
2469:
2468:
2466:
2465:
2464:
2458:
2456:
2454:
2450:
2446:
2442:
2438:
2434:
2430:
2426:
2422:
2414:
2412:
2408:
2404:
2397:
2393:
2387:
2384:
2378:
2376:
2372:
2368:
2364:
2360:
2356:
2352:
2344:
2342:
2330:
2328:
2324:
2320:
2315:
2311:
2307:
2303:
2297:
2295:
2291:
2287:
2279:
2277:
2275:
2271:
2267:
2266:peroxynitrite
2261:
2258:
2254:
2250:
2246:
2241:
2234:
2232:
2230:
2226:
2222:
2218:
2213:
2211:
2206:
2203:
2199:
2194:
2190:
2187:
2183:
2179:
2177:
2173:
2169:
2168:
2163:
2162:
2157:
2153:
2149:
2148:
2143:
2142:isoflavonoids
2139:
2135:
2128:
2127:
2122:
2118:
2111:
2106:
2102:
2098:
2096:
2095:
2090:
2087:
2084:
2083:
2082:
2075:
2072:
2069:
2066:
2063:
2060:
2057:
2056:
2053:
2050:
2047:
2044:
2041:
2038:
2035:
2034:
2031:
2028:
2025:
2022:
2019:
2016:
2013:
2012:
2009:
2006:
2003:
2000:
1997:
1994:
1991:
1985:
1982:
1979:
1976:
1973:
1971:
1968:
1965:
1962:
1961:
1957:
1954:
1952:
1949:
1946:
1943:
1942:
1939:
1936:
1933:
1930:
1928:
1925:
1922:
1919:
1918:
1915:
1912:
1909:
1906:
1904:
1901:
1898:
1895:
1894:
1891:
1888:
1885:
1882:
1880:
1877:
1875:NDUFC1 / KFYI
1874:
1871:
1870:
1867:
1864:
1861:
1858:
1856:
1853:
1851:NDUFB1 / MNLL
1850:
1847:
1846:
1843:
1840:
1837:
1834:
1832:
1829:
1827:NDUFB5 / SGDH
1826:
1823:
1822:
1819:
1816:
1813:
1810:
1808:
1805:
1803:NDUFA4 / MLRQ
1802:
1799:
1798:
1795:
1792:
1789:
1786:
1784:
1781:
1778:
1775:
1774:
1771:
1768:
1765:
1762:
1760:
1757:
1754:
1751:
1750:
1747:
1744:
1741:
1738:
1736:
1733:
1731:NDUFB2 / AGGG
1730:
1727:
1726:
1723:
1720:
1717:
1714:
1712:
1709:
1706:
1703:
1702:
1699:
1696:
1693:
1690:
1688:
1685:
1683:NDUFB8 / ASHI
1682:
1679:
1678:
1675:
1672:
1669:
1666:
1664:
1661:
1658:
1655:
1654:
1651:
1648:
1645:
1642:
1640:
1637:
1634:
1631:
1630:
1627:
1624:
1621:
1618:
1616:
1613:
1611:NDUFA8 / PGIV
1610:
1607:
1606:
1603:
1600:
1597:
1594:
1592:
1589:
1586:
1583:
1582:
1579:
1576:
1573:
1570:
1568:
1565:
1562:
1559:
1558:
1555:
1552:
1549:
1546:
1544:
1541:
1538:
1535:
1534:
1531:
1528:
1525:
1522:
1520:
1517:
1515:NDUFS5 / PFFD
1514:
1511:
1510:
1507:
1504:
1501:
1498:
1496:
1493:
1490:
1487:
1486:
1483:
1480:
1477:
1474:
1472:
1469:
1466:
1463:
1462:
1459:
1456:
1453:
1450:
1448:
1445:
1442:
1439:
1438:
1435:
1432:
1429:
1426:
1424:
1421:
1419:NDUFA5 / AB13
1418:
1415:
1414:
1411:
1408:
1405:
1402:
1400:
1397:
1394:
1391:
1390:
1387:
1384:
1381:
1378:
1376:
1373:
1371:NDUFA1 / MFWE
1370:
1367:
1366:
1363:
1360:
1357:
1354:
1352:
1349:
1346:
1343:
1342:
1339:
1336:
1333:
1330:
1328:
1325:
1322:
1319:
1318:
1315:
1312:
1309:
1306:
1304:
1301:
1298:
1295:
1294:
1291:
1288:
1285:
1282:
1280:
1277:
1275:NDUFS4 / AQDQ
1274:
1271:
1270:
1267:
1264:
1261:
1258:
1256:
1253:
1250:
1247:
1246:
1243:
1240:
1237:
1234:
1232:
1229:
1226:
1223:
1222:
1217:
1214:
1211:
1208:
1206:
1203:
1199:
1197:
1194:
1191:
1188:
1187:
1184:
1181:
1177:
1174:
1170:
1167:
1164:
1162:
1159:
1155:
1153:
1150:
1147:
1144:
1143:
1140:
1137:
1134:
1132:
1129:
1125:
1123:
1120:
1117:
1114:
1113:
1110:
1107:
1103:
1100:
1097:
1095:
1092:
1088:
1086:
1083:
1080:
1077:
1076:
1073:
1070:
1067:
1065:
1062:
1058:
1056:
1053:
1050:
1047:
1046:
1043:
1040:
1036:
1033:
1030:
1028:
1025:
1021:
1019:
1016:
1013:
1010:
1009:
1006:
1003:
1000:
998:
995:
991:
989:
986:
983:
980:
979:
976:
973:
970:
968:
965:
962:
959:
955:
953:
950:
947:
944:
943:
940:
937:
934:
932:
929:
926:
923:
919:
917:
914:
911:
908:
907:
904:
901:
898:
896:
893:
890:
887:
883:
881:
878:
875:
872:
871:
868:
865:
862:
860:
857:
854:
851:
847:
845:
842:
839:
836:
835:
832:
829:
826:
824:
821:
818:
815:
811:
809:
806:
803:
800:
799:
796:
793:
790:
788:
785:
782:
779:
775:
773:
770:
767:
764:
763:
760:
757:
754:
752:
749:
746:
743:
739:
737:
734:
731:
728:
727:
722:
718:
715:
712:
708:
705:
702:
698:
695:
692:
691:
685:
683:
678:
674:
670:
668:
663:
659:
653:
651:
647:
643:
640:
636:
628:
626:
619:
615:
610:
608:
604:
600:
596:
592:
586:
584:
580:
576:
567:
565:
561:
554:
552:
546:
542:
538:
534:
530:
522:
514:
509:
507:
505:
500:
498:
494:
490:
485:
483:
479:
474:
470:
468:
464:
460:
459:
454:
450:
449:
444:
440:
439:
434:
430:
426:
422:
403:
402:
401:
398:
396:
392:
388:
375:
363:
355:
348:
346:
344:
340:
336:
322:
319:
317:
313:
310:
307:
305:
301:
298:
295:
293:
289:
284:
280:
277:
273:
270:
268:
267:Gene Ontology
264:
261:
258:
255:
252:
249:
245:
242:
239:
237:
233:
230:
227:
225:
221:
218:
215:
213:
209:
206:
205:NiceZyme view
203:
201:
197:
194:
191:
189:
185:
182:
179:
177:
173:
168:
165:
162:
160:
156:
151:
144:
141:
139:
135:
132:
129:
127:
123:
120:
117:
115:
111:
107:
103:
98:
93:
90:
88:
87:mitochondrial
84:
80:
76:
72:
68:
64:
60:
56:
52:
49:
45:
32:
19:
7559:
7549:
7548:
7510:Translocases
7507:
7494:
7481:
7468:
7455:
7445:Transferases
7442:
7429:
7286:Binding site
7223:
7070:ATP synthase
6877:
6845:
6813:
6761:
6728:ATP synthase
6708:
6676:
6649:
6627:
6607:Cytochrome c
6590:
6530:
6520:Kynureninase
6507:
6480:
6365:proton pumps
6236:
6225:Cytochrome c
6133:succinyl-CoA
5888:Cryptochrome
5742:
5738:
5732:
5724:
5699:
5695:
5689:
5662:
5658:
5648:
5624:(4): 360â8.
5621:
5617:
5607:
5572:
5568:
5520:
5516:
5506:
5473:
5469:
5463:
5436:
5432:
5422:
5387:
5383:
5373:
5346:
5342:
5332:
5287:
5283:
5273:
5246:
5243:Biochemistry
5242:
5232:
5197:
5193:
5183:
5161:(2): 491â9.
5158:
5154:
5147:
5112:
5108:
5060:
5056:
5046:
5016:(1): 89â95.
5013:
5009:
5003:
4968:
4964:
4918:
4914:
4908:
4873:
4869:
4859:
4824:
4820:
4810:
4783:
4779:
4769:
4734:
4730:
4720:
4685:
4681:
4671:
4634:
4624:
4599:
4595:
4589:
4554:
4550:
4540:
4513:
4509:
4499:
4466:
4462:
4456:
4421:
4411:
4379:(5): 883â8.
4376:
4373:FEBS Letters
4372:
4362:
4337:
4333:
4327:
4300:
4296:
4286:
4251:
4247:
4199:
4195:
4185:
4150:
4146:
4136:
4101:
4097:
4087:
4060:
4056:
4046:
4001:
3997:
3987:
3962:
3958:
3952:
3925:
3921:
3911:
3884:
3880:
3870:
3829:
3825:
3815:
3772:
3768:
3755:
3728:
3724:
3714:
3687:
3683:
3673:
3649:Biochemistry
3648:
3640:
3599:
3595:
3589:
3556:
3552:
3539:
3512:
3508:
3498:
3473:
3469:
3459:
3422:
3418:
3404:
3367:
3363:
3353:
3326:
3322:
3313:Efremov RG,
3270:
3267:Biochemistry
3266:
3256:
3229:
3225:
3215:
3196:
3190:
3157:
3153:
3105:
3101:
3094:
3067:
3063:
3053:
3018:
3015:Biochemistry
3014:
3004:
2977:
2973:
2925:
2922:FEBS Letters
2921:
2911:
2884:
2880:
2870:
2846:(2): 300â4.
2843:
2840:FEBS Letters
2839:
2829:
2804:
2800:
2794:
2786:Biochemistry
2785:
2462:
2452:
2448:
2444:
2432:
2424:
2418:
2409:
2405:
2388:
2379:
2353:, including
2348:
2331:
2323:oxaloacetate
2298:
2292:(as well as
2283:
2274:nitric oxide
2262:
2242:
2238:
2231:catabolism.
2214:
2207:
2195:
2191:
2180:
2176:piericidin A
2165:
2159:
2156:Lonchocarpus
2145:
2124:
2115:
2092:
2080:
1635:NDUFB9 / B22
1587:NDUFB7 / B18
1563:NDUFA13 /A13
1539:NDUFB4 / B15
1443:NDUFA6 / B14
1395:NDUFB3 / B12
1227:NDUFS6 / 13A
679:
675:
671:
661:
657:
654:
632:
611:
587:
571:
562:
558:
536:
533:coenzyme Q10
518:
504:ATP synthase
501:
488:
486:
475:
471:
466:
462:
456:
452:
446:
436:
418:
408:â NAD + CoQH
399:
384:
357:NAD to NADH.
331:
193:BRENDA entry
83:coenzyme Q10
62:
58:
54:
43:
42:
7281:Active site
7020:Complex III
6242:synthesis:
6059:Anaplerotic
4971:(1): 1â13.
4827:: 517â524.
4635:GeroScience
3070:(1): 3â11.
2441:angiosperms
2437:gnetophytes
2429:gymnosperms
2421:land plants
2371:complex III
2182:Acetogenins
2147:Loganiaceae
2064:NDUF4_HUMAN
2042:NDUF3_HUMAN
2020:MIMIT_HUMAN
1998:CIA30_HUMAN
1970:NDUB6_HUMAN
1951:NDUV3_HUMAN
1927:NUA4L_HUMAN
1903:NDUAA_HUMAN
1879:NDUC1_HUMAN
1855:NDUB1_HUMAN
1831:NDUB5_HUMAN
1807:NDUA4_HUMAN
1783:NDUA3_HUMAN
1779:NDUFA3 / B9
1759:NDUA7_HUMAN
1735:NDUB2_HUMAN
1711:NDUC2_HUMAN
1687:NDUB8_HUMAN
1663:NDUBA_HUMAN
1639:NDUB9_HUMAN
1615:NDUA8_HUMAN
1591:NDUB7_HUMAN
1567:NDUAD_HUMAN
1543:NDUB4_HUMAN
1519:NDUS5_HUMAN
1495:NDUBB_HUMAN
1471:NDUAB_HUMAN
1447:NDUA6_HUMAN
1423:NDUA5_HUMAN
1399:NDUB3_HUMAN
1375:NDUA1_HUMAN
1351:NDUA2_HUMAN
1347:NDUFA2 / B8
1303:NDUA9_HUMAN
1279:NDUS4_HUMAN
1255:NDUAC_HUMAN
1231:NDUS6_HUMAN
1122:NU4LM_HUMAN
1118:ND4L / NULM
952:NDUS1_HUMAN
916:NDUV1_HUMAN
880:NDUS2_HUMAN
844:NDUS3_HUMAN
808:NDUV2_HUMAN
772:NDUS8_HUMAN
736:NDUS7_HUMAN
603:semiquinone
599:semiquinone
377:CoQ to CoQH
365:FMN to FMNH
181:IntEnz view
153:Identifiers
126:OPM protein
100:Identifiers
7587:Glycolysis
7576:Categories
7484:Isomerases
7458:Hydrolases
7325:Regulation
7040:Complex IV
6847:urea cycle
6535:metabolism
6511:metabolism
6509:tryptophan
6237:Coenzyme Q
6211:Coenzyme Q
6069:acetyl-CoA
5985:Metabolism
5908:Flavodoxin
5794:(Requires
5696:Toxicology
3822:Sazanov LA
3761:Sazanov LA
3466:Sazanov LA
3411:Sazanov LA
3315:Sazanov LA
2769:References
2383:proteasome
2375:superoxide
2290:superoxide
2225:nefazodone
2186:Annonaceae
2172:ubiquinone
2121:acetogenin
2117:Bullatacin
2112:Inhibitors
2105:complex IV
1327:ACPM_HUMAN
1196:NU6M_HUMAN
1192:ND6 / NU6M
1152:NU5M_HUMAN
1148:ND5 / NU5M
1085:NU4M_HUMAN
1081:ND4 / NU4M
1055:NU3M_HUMAN
1051:ND3 / NU3M
1018:NU2M_HUMAN
1014:ND2 / NU2M
988:NU1M_HUMAN
984:ND1 / NU1M
618:antiporter
541:Ubiquinone
467:R. marinus
250:structures
217:KEGG entry
138:Membranome
7552:this edit
7363:EC number
7224:see also
6970:Complex I
6778:Aconitase
6011:Aconitase
5796:MDL Chime
5498:207990089
4653:2509-2723
4270:1932-7420
2807:: 69â92.
2392:succinate
2345:Pathology
2319:succinate
2243:The high
2221:flutamide
2210:Metformin
2161:Faboideae
2123:found in
1698:PF05821
614:ubiquinol
607:ubiquinol
595:ubiquinol
545:ubiquinol
510:Mechanism
493:TC# 3.D.1
478:apoptosis
170:Databases
75:electrons
7592:EC 7.1.1
7387:Kinetics
7311:Cofactor
7274:Activity
6924:Frataxin
6798:Fumarase
6464:proteins
6420:V-ATPase
6361:Ion pump
6043:Fumarase
5771:34830386
5716:20132858
5681:20153825
5640:20368511
5599:31037949
5547:29643256
5490:31760927
5455:20132468
5414:20417232
5365:15985426
5324:16682634
5265:18307315
5224:29211056
5175:17916065
5139:28914132
5087:30582748
4995:19061483
4943:29513174
4935:11994742
4900:29629602
4851:30037775
4802:17956863
4761:26777588
4712:24569053
4662:11226580
4616:21718246
4581:22117616
4483:18599602
4403:20074573
4278:22902835
4226:19463981
4177:17557076
4128:16200211
4079:21749854
4038:20133838
3979:15843018
3944:15450959
3903:20493164
3854:20505720
3799:16469879
3747:22902835
3706:16950771
3624:15988517
3581:23582849
3573:25554780
3531:21565159
3490:17360196
3451:19635800
3396:21659507
3345:22386882
3297:22122402
3182:31633494
3174:25991374
3130:25828432
3122:19834734
3086:20371875
3045:17760425
2996:21172303
2944:10371157
2903:17094937
2821:16756485
2396:transfer
2249:cysteine
2167:Fabaceae
2138:rotenone
2074:PF06784
2052:PF05071
2030:PF05071
2008:PF08547
1981:PF09782
1938:PF15880
1923:NDUFA4L2
1914:PF01712
1890:PF15088
1866:PF08040
1818:PF06522
1770:PF07347
1746:PF14813
1650:PF05347
1626:PF06747
1602:PF05676
1530:PF10200
1458:PF05347
1410:PF08122
1386:PF15879
1314:PF01370
1242:PF10276
1213:PF00499
1183:PF00662
967:1.6.99.3
931:1.6.99.3
895:1.6.99.3
859:1.6.99.3
823:1.6.99.3
787:1.6.99.3
751:1.6.99.3
703:subunit
349:Function
337:damage (
321:proteins
309:articles
297:articles
254:RCSB PDB
7543:Biology
7497:Ligases
7267:Enzymes
7084:MT-ATP8
7079:MT-ATP6
6999:MT-ND4L
6215:(CoQ10)
6194:Primary
6050:and ETC
5992:enzymes
5853:Protein
5762:8621559
5590:6657304
5538:5916474
5405:2885979
5315:1472492
5292:Bibcode
5215:5866163
5130:5718331
5078:7086484
5038:7501110
5030:9067806
4986:2605959
4891:6727140
4842:6389362
4752:4893024
4703:4331042
4572:3398862
4532:9230920
4491:1844073
4448:8037664
4439:1137156
4394:2836797
4354:7132401
4319:9711297
4217:2694978
4168:1914096
4119:1236688
4029:2808219
4006:Bibcode
3862:4372778
3834:Bibcode
3807:1892332
3777:Bibcode
3769:Science
3632:4372674
3604:Bibcode
3553:Science
3442:2785608
3387:3149303
3288:3254188
3248:9593887
3036:2258335
2952:2337382
2862:6325245
2748:MT-ND4L
2620:NDUFB11
2614:NDUFB10
2549:NDUFAF1
2540:NDUFA13
2534:NDUFA12
2525:NDUFA10
2270:hypoxia
2229:glucose
2081:Notes:
2061:NDUFAF4
2039:NDUFAF3
2017:NDUFAF2
1995:NDUFAF1
1842:PF09781
1794:PF14987
1722:PF06374
1674:PF10249
1578:PF06212
1554:PF07225
1506:PF10183
1482:PF02466
1434:PF04716
1362:PF05047
1338:PF00550
1290:PF04800
1266:PF05071
1205:1.6.5.3
1176:PF06455
1169:PF00361
1161:1.6.5.3
1139:PF00420
1131:1.6.5.3
1109:PF00361
1102:PF01059
1094:1.6.5.3
1072:PF00507
1064:1.6.5.3
1042:PF06444
1035:PF00361
1027:1.6.5.3
1005:PF00146
997:1.6.5.3
975:PF00384
961:1.6.5.3
939:PF01512
925:1.6.5.3
903:PF00346
889:1.6.5.3
867:PF00329
853:1.6.5.3
831:PF01257
817:1.6.5.3
795:PF12838
781:1.6.5.3
759:PF01058
745:1.6.5.3
711:UniProt
658:E. coli
635:mammals
453:E. coli
421:protons
276:QuickGO
241:profile
224:MetaCyc
164:7.1.1.2
69:of the
51:7.1.1.2
7550:As of
7529:Portal
7471:Lyases
7189:MT-TS2
7184:MT-TS1
7154:MT-TL2
7149:MT-TL1
7059:MT-CO3
7054:MT-CO2
7049:MT-CO1
7029:MT-CYB
7009:MT-ND6
7004:MT-ND5
6994:MT-ND4
6989:MT-ND3
6984:MT-ND2
6979:MT-ND1
6752:Matrix
6284:COQ10B
6279:COQ10A
5818:(MeSH)
5769:
5759:
5735:Genes"
5714:
5679:
5638:
5597:
5587:
5545:
5535:
5517:Stroke
5496:
5488:
5453:
5412:
5402:
5363:
5322:
5312:
5263:
5222:
5212:
5173:
5137:
5127:
5085:
5075:
5036:
5028:
4993:
4983:
4941:
4933:
4898:
4888:
4849:
4839:
4800:
4759:
4749:
4710:
4700:
4659:
4651:
4614:
4579:
4569:
4530:
4489:
4481:
4446:
4436:
4401:
4391:
4352:
4317:
4276:
4268:
4224:
4214:
4175:
4165:
4126:
4116:
4077:
4036:
4026:
3977:
3942:
3901:
3860:
3852:
3826:Nature
3805:
3797:
3745:
3704:
3661:
3657:â826.
3630:
3622:
3596:Nature
3579:
3571:
3529:
3488:
3449:
3439:
3394:
3384:
3343:
3295:
3285:
3246:
3203:
3180:
3172:
3128:
3120:
3102:Planta
3084:
3043:
3033:
2994:
2950:
2942:
2901:
2860:
2819:
2760:MT-ND6
2754:MT-ND5
2742:MT-ND4
2736:MT-ND3
2730:MT-ND2
2724:MT-ND1
2713:NDUFV3
2707:NDUFV2
2701:NDUFV1
2690:NDUFS8
2684:NDUFS7
2678:NDUFS6
2672:NDUFS5
2666:NDUFS4
2660:NDUFS3
2654:NDUFS2
2648:NDUFS1
2637:NDUFC2
2631:NDUFC1
2608:NDUFB9
2602:NDUFB8
2596:NDUFB7
2590:NDUFB6
2575:NDUFB2
2569:NDUFB1
2519:NDUFA9
2513:NDUFA8
2504:NDUFA6
2498:NDUFA5
2486:NDUFA4
2477:NDUFA2
2471:NDUFA1
2327:malate
2152:Derris
2101:NDUFA4
1966:NDUFB6
1947:NDUFV3
701:Bovine
639:flavin
339:stroke
304:PubMed
286:Search
272:AmiGO
260:PDBsum
200:ExPASy
188:BRENDA
176:IntEnz
159:EC no.
105:Symbol
7423:Types
7209:MT-TY
7204:MT-TW
7199:MT-TV
7194:MT-TT
7179:MT-TR
7174:MT-TQ
7169:MT-TP
7164:MT-TN
7159:MT-TM
7144:MT-TK
7139:MT-TI
7134:MT-TH
7129:MT-TG
7124:MT-TF
7119:MT-TE
7114:MT-TD
7109:MT-TC
7104:MT-TA
6900:PMPCB
6887:ALDH2
6709:other
6403:Other
6369:TC 3D
6303:Other
6294:PDSS2
6289:PDSS1
5999:Cycle
5494:S2CID
5034:S2CID
4939:S2CID
4487:S2CID
3858:S2CID
3803:S2CID
3628:S2CID
3577:S2CID
3549:(PDF)
3178:S2CID
3126:S2CID
2948:S2CID
2459:Genes
2433:Pinus
2399:(FMNH
2359:mtDNA
2184:from
697:Human
547:(CoQH
236:PRIAM
77:from
35:(CoQH
7566:GFDL
7515:list
7508:EC7
7502:list
7495:EC6
7489:list
7482:EC5
7476:list
7469:EC4
7463:list
7456:EC3
7450:list
7443:EC2
7437:list
7430:EC1
7095:tRNA
6274:COQ9
6269:COQ7
6264:COQ6
6259:COQ5
6254:COQ4
6249:COQ3
6244:COQ2
6100:and
6037:SDHA
5767:PMID
5712:PMID
5677:PMID
5663:1802
5636:PMID
5595:PMID
5543:PMID
5486:PMID
5451:PMID
5410:PMID
5361:PMID
5320:PMID
5261:PMID
5220:PMID
5171:PMID
5135:PMID
5083:PMID
5026:PMID
4991:PMID
4931:PMID
4896:PMID
4847:PMID
4798:PMID
4757:PMID
4735:1857
4708:PMID
4686:1837
4649:ISSN
4612:PMID
4577:PMID
4528:PMID
4514:1320
4479:PMID
4444:PMID
4399:PMID
4350:PMID
4315:PMID
4301:1365
4274:PMID
4266:ISSN
4222:PMID
4173:PMID
4124:PMID
4075:PMID
4061:1807
4034:PMID
3975:PMID
3940:PMID
3926:1658
3899:PMID
3885:1797
3850:PMID
3795:PMID
3743:PMID
3702:PMID
3659:ISBN
3620:PMID
3569:PMID
3527:PMID
3513:1807
3486:PMID
3447:PMID
3392:PMID
3341:PMID
3327:1817
3293:PMID
3244:PMID
3230:1364
3201:ISBN
3170:PMID
3118:PMID
3082:PMID
3041:PMID
2992:PMID
2978:1807
2940:PMID
2899:PMID
2885:1757
2858:PMID
2817:PMID
2435:and
2333:FMNH
2257:DTNB
2223:and
2154:and
2119:(an
2071:Pfam
2049:Pfam
2027:Pfam
2005:Pfam
1978:Pfam
1935:Pfam
1911:Pfam
1887:Pfam
1863:Pfam
1839:Pfam
1815:Pfam
1791:Pfam
1767:Pfam
1743:Pfam
1719:Pfam
1695:Pfam
1671:Pfam
1647:Pfam
1623:Pfam
1599:Pfam
1575:Pfam
1551:Pfam
1527:Pfam
1503:Pfam
1479:Pfam
1455:Pfam
1431:Pfam
1407:Pfam
1383:Pfam
1359:Pfam
1335:Pfam
1311:Pfam
1287:Pfam
1263:Pfam
1239:Pfam
1210:Pfam
1180:Pfam
1173:Pfam
1166:Pfam
1136:Pfam
1106:Pfam
1099:Pfam
1069:Pfam
1039:Pfam
1032:Pfam
1002:Pfam
972:Pfam
936:Pfam
900:Pfam
864:Pfam
828:Pfam
792:Pfam
756:Pfam
717:Pfam
605:and
581:and
575:heme
425:NADH
412:+ 4H
341:and
316:NCBI
257:PDBe
212:KEGG
131:6g72
79:NADH
61:and
6377:ETC
6150:to
6131:to
6112:to
6067:to
5757:PMC
5747:doi
5733:ndh
5704:doi
5700:270
5667:doi
5626:doi
5585:PMC
5577:doi
5533:PMC
5525:doi
5478:doi
5441:doi
5437:113
5400:PMC
5392:doi
5351:doi
5347:280
5310:PMC
5300:doi
5288:103
5251:doi
5210:PMC
5202:doi
5163:doi
5159:409
5125:PMC
5117:doi
5073:PMC
5065:doi
5061:148
5018:doi
4981:PMC
4973:doi
4969:417
4923:doi
4886:PMC
4878:doi
4837:PMC
4829:doi
4825:124
4788:doi
4784:282
4747:PMC
4739:doi
4698:PMC
4690:doi
4657:PMC
4639:doi
4604:doi
4567:PMC
4559:doi
4555:122
4518:doi
4471:doi
4434:PMC
4426:doi
4389:PMC
4381:doi
4377:584
4342:doi
4305:doi
4256:doi
4212:PMC
4204:doi
4163:PMC
4155:doi
4114:PMC
4106:doi
4102:115
4065:doi
4024:PMC
4014:doi
4002:107
3967:doi
3963:348
3930:doi
3889:doi
3842:doi
3830:465
3785:doi
3773:311
3733:doi
3692:doi
3688:281
3655:813
3612:doi
3600:435
3561:doi
3557:347
3517:doi
3478:doi
3474:159
3437:PMC
3427:doi
3423:284
3382:PMC
3372:doi
3368:286
3331:doi
3283:PMC
3275:doi
3234:doi
3162:doi
3110:doi
3106:231
3072:doi
3031:PMC
3023:doi
2982:doi
2930:doi
2926:451
2889:doi
2848:doi
2844:169
2809:doi
2453:ndh
2449:ndh
2445:ndh
2425:ndh
2377:).
2325:or
2312:or
2255:or
2150:),
662:nuo
579:III
551:).
529:FAD
433:ATP
414:out
292:PMC
248:PDB
143:255
119:246
81:to
7578::
6363::
6239:10
6213:10
6089:E3
6087:,
6085:E2
6083:,
6081:E1
5987::
5855::
5765:.
5755:.
5743:22
5741:.
5737:.
5710:.
5698:.
5675:.
5661:.
5657:.
5634:.
5622:67
5620:.
5616:.
5593:.
5583:.
5573:31
5571:.
5567:.
5555:^
5541:.
5531:.
5521:49
5519:.
5515:.
5492:.
5484:.
5474:84
5472:.
5449:.
5435:.
5431:.
5408:.
5398:.
5388:31
5386:.
5382:.
5359:.
5345:.
5341:.
5318:.
5308:.
5298:.
5286:.
5282:.
5259:.
5247:47
5245:.
5241:.
5218:.
5208:.
5198:83
5196:.
5192:.
5169:.
5157:.
5133:.
5123:.
5113:37
5111:.
5107:.
5095:^
5081:.
5071:.
5059:.
5055:.
5032:.
5024:.
5014:29
5012:.
4989:.
4979:.
4967:.
4963:.
4951:^
4937:.
4929:.
4917:.
4894:.
4884:.
4874:39
4872:.
4868:.
4845:.
4835:.
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