Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. (Homo sapiens)

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12-1410, 113-810, 11991, 210, 1110, 11910, 11mitochondrial intermembrane spacemitochondrial matrixATPubiquinolFatty Acid anion "head-in"Purine nucleotideATPase complexFA anion:UCP dimer "head-in" complexFA anion:UCP dimer "head-out" complexOxygenubiquinoneubiquinoneLong-chain fatty acidubiquinolADPATPase-ATP complexH2OUCP dimerSuccinate dehydrogenase complex (reduced)Complex I - NADH:Ubiquinone oxidoreductaseETF (reduced)Fatty Acid "head-out"Cytochrome c (reduced)Cytochrome c (oxidized)UCP dimerH+Ubiquinol-cytochrome c reductaseFatty Acid "head-in"Cytochrome c oxidaseSuccinate dehydrogenase complex (oxidised)NAD+ubiquinolFADH2Fatty Acid anion "head-out"FADETFDHETFOrthophosphateH+H2ONADHATPase-ADP and Pi complex


Description

Oxidation of fatty acids and pyruvate in the mitochondrial matrix yield large amounts of NADH. The respiratory electron transport chain couples the re-oxidation of this NADH to NAD+ to the export of protons from the mitochonrial matrix, generating a chemiosmotic gradient across the inner mitochondrial membrane. This gradient is used to drive the synthesis of ATP; it can also be bypassed by uncoupling proteins to generate heat, a reaction in brown fat that may be important in regulation of body temperature in newborn children.

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Bibliography

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  1. Stanley CA, Hale DE.; ''Genetic disorders of mitochondrial fatty acid oxidation.''; PubMed Europe PMC Scholia
  2. Allan CM, Hill S, Morvaridi S, Saiki R, Johnson JS, Liau WS, Hirano K, Kawashima T, Ji Z, Loo JA, Shepherd JN, Clarke CF.; ''A conserved START domain coenzyme Q-binding polypeptide is required for efficient Q biosynthesis, respiratory electron transport, and antioxidant function in Saccharomyces cerevisiae.''; PubMed Europe PMC Scholia
  3. Yoshida S, Tsutsumi S, Muhlebach G, Sourbier C, Lee MJ, Lee S, Vartholomaiou E, Tatokoro M, Beebe K, Miyajima N, Mohney RP, Chen Y, Hasumi H, Xu W, Fukushima H, Nakamura K, Koga F, Kihara K, Trepel J, Picard D, Neckers L.; ''Molecular chaperone TRAP1 regulates a metabolic switch between mitochondrial respiration and aerobic glycolysis.''; PubMed Europe PMC Scholia
  4. Fontanesi F, Soto IC, Horn D, Barrientos A.; ''Assembly of mitochondrial cytochrome c-oxidase, a complicated and highly regulated cellular process.''; PubMed Europe PMC Scholia
  5. Boyer PD, Cross RL, Momsen W.; ''A new concept for energy coupling in oxidative phosphorylation based on a molecular explanation of the oxygen exchange reactions.''; PubMed Europe PMC Scholia
  6. Hirst J, Carroll J, Fearnley IM, Shannon RJ, Walker JE.; ''The nuclear encoded subunits of complex I from bovine heart mitochondria.''; PubMed Europe PMC Scholia
  7. Barros MH, Johnson A, Gin P, Marbois BN, Clarke CF, Tzagoloff A.; ''The Saccharomyces cerevisiae COQ10 gene encodes a START domain protein required for function of coenzyme Q in respiration.''; PubMed Europe PMC Scholia
  8. Balsa E, Marco R, Perales-Clemente E, Szklarczyk R, Calvo E, Landázuri MO, Enríquez JA.; ''NDUFA4 is a subunit of complex IV of the mammalian electron transport chain.''; PubMed Europe PMC Scholia
  9. Mitchell P.; ''Protonmotive redox mechanism of the cytochrome b-c1 complex in the respiratory chain: protonmotive ubiquinone cycle.''; PubMed Europe PMC Scholia
  10. Wikstrom MK.; ''Proton pump coupled to cytochrome c oxidase in mitochondria.''; PubMed Europe PMC Scholia
  11. Yano T.; ''The energy-transducing NADH: quinone oxidoreductase, complex I.''; PubMed Europe PMC Scholia
  12. Wood PA.; ''Defects in mitochondrial beta-oxidation of fatty acids.''; PubMed Europe PMC Scholia
  13. Loeffen J, Elpeleg O, Smeitink J, Smeets R, Stöckler-Ipsiroglu S, Mandel H, Sengers R, Trijbels F, van den Heuvel L.; ''Mutations in the complex I NDUFS2 gene of patients with cardiomyopathy and encephalomyopathy.''; PubMed Europe PMC Scholia
  14. Belogrudov GI, Hatefi Y.; ''Factor B and the mitochondrial ATP synthase complex.''; PubMed Europe PMC Scholia
  15. Rinaldo P, Matern D, Bennett MJ.; ''Fatty acid oxidation disorders.''; PubMed Europe PMC Scholia
  16. Sciacovelli M, Guzzo G, Morello V, Frezza C, Zheng L, Nannini N, Calabrese F, Laudiero G, Esposito F, Landriscina M, Defilippi P, Bernardi P, Rasola A.; ''The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenase.''; PubMed Europe PMC Scholia
  17. Trumpower BL, Gennis RB.; ''Energy transduction by cytochrome complexes in mitochondrial and bacterial respiration: the enzymology of coupling electron transfer reactions to transmembrane proton translocation.''; PubMed Europe PMC Scholia
  18. Stiburek L, Hansikova H, Tesarova M, Cerna L, Zeman J.; ''Biogenesis of eukaryotic cytochrome c oxidase.''; PubMed Europe PMC Scholia
  19. Schultz BE, Chan SI.; ''Structures and proton-pumping strategies of mitochondrial respiratory enzymes.''; PubMed Europe PMC Scholia
  20. Garlid KD, Jaburek M, Jezek P.; ''Mechanism of uncoupling protein action.''; PubMed Europe PMC Scholia
  21. Belogrudov GI.; ''Factor B is essential for ATP synthesis by mitochondria.''; PubMed Europe PMC Scholia
  22. Roe CR, Roe DS.; ''Recent developments in the investigation of inherited metabolic disorders using cultured human cells.''; PubMed Europe PMC Scholia
  23. Loeffen JL, Triepels RH, van den Heuvel LP, Schuelke M, Buskens CA, Smeets RJ, Trijbels JM, Smeitink JA.; ''cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed.''; PubMed Europe PMC Scholia
  24. Long JZ, Svensson KJ, Bateman LA, Lin H, Kamenecka T, Lokurkar IA, Lou J, Rao RR, Chang MR, Jedrychowski MP, Paulo JA, Gygi SP, Griffin PR, Nomura DK, Spiegelman BM.; ''The Secreted Enzyme PM20D1 Regulates Lipidated Amino Acid Uncouplers of Mitochondria.''; PubMed Europe PMC Scholia
  25. Estornell E, Fato R, Castelluccio C, Cavazzoni M, Parenti Castelli G, Lenaz G.; ''Saturation kinetics of coenzyme Q in NADH and succinate oxidation in beef heart mitochondria.''; PubMed Europe PMC Scholia
  26. Soto IC, Fontanesi F, Liu J, Barrientos A.; ''Biogenesis and assembly of eukaryotic cytochrome c oxidase catalytic core.''; PubMed Europe PMC Scholia
  27. Echtay KS, Roussel D, St-Pierre J, Jekabsons MB, Cadenas S, Stuart JA, Harper JA, Roebuck SJ, Morrison A, Pickering S, Clapham JC, Brand MD.; ''Superoxide activates mitochondrial uncoupling proteins.''; PubMed Europe PMC Scholia
  28. Kevelam SH, Rodenburg RJ, Wolf NI, Ferreira P, Lunsing RJ, Nijtmans LG, Mitchell A, Arroyo HA, Rating D, Vanderver A, van Berkel CG, Abbink TE, Heutink P, van der Knaap MS.; ''NUBPL mutations in patients with complex I deficiency and a distinct MRI pattern.''; PubMed Europe PMC Scholia
  29. Schuelke M, Loeffen J, Mariman E, Smeitink J, van den Heuvel L.; ''Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3'UTR with the 5'UTR of the gamma-interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation?''; PubMed Europe PMC Scholia
  30. Garlid KD, Orosz DE, Modrianský M, Vassanelli S, Jezek P.; ''On the mechanism of fatty acid-induced proton transport by mitochondrial uncoupling protein.''; PubMed Europe PMC Scholia
  31. Pitceathly RD, Rahman S, Wedatilake Y, Polke JM, Cirak S, Foley AR, Sailer A, Hurles ME, Stalker J, Hargreaves I, Woodward CE, Sweeney MG, Muntoni F, Houlden H, Taanman JW, Hanna MG, UK10K Consortium.; ''NDUFA4 mutations underlie dysfunction of a cytochrome c oxidase subunit linked to human neurological disease.''; PubMed Europe PMC Scholia
  32. Smeitink J, Sengers R, Trijbels F, van den Heuvel L.; ''Human NADH:ubiquinone oxidoreductase.''; PubMed Europe PMC Scholia
  33. Mckenzie M, Ryan MT.; ''Assembly factors of human mitochondrial complex I and their defects in disease.''; PubMed Europe PMC Scholia
  34. Andrews B, Carroll J, Ding S, Fearnley IM, Walker JE.; ''Assembly factors for the membrane arm of human complex I.''; PubMed Europe PMC Scholia
  35. Jezek P, Hanus J, Semrad C, Garlid KD.; ''Photoactivated azido fatty acid irreversibly inhibits anion and proton transport through the mitochondrial uncoupling protein.''; PubMed Europe PMC Scholia
  36. Bourges I, Ramus C, Mousson de Camaret B, Beugnot R, Remacle C, Cardol P, Hofhaus G, Issartel JP.; ''Structural organization of mitochondrial human complex I: role of the ND4 and ND5 mitochondria-encoded subunits and interaction with prohibitin.''; PubMed Europe PMC Scholia
  37. Mimaki M, Wang X, McKenzie M, Thorburn DR, Ryan MT.; ''Understanding mitochondrial complex I assembly in health and disease.''; PubMed Europe PMC Scholia
  38. Sass JO.; ''Inborn errors of ketogenesis and ketone body utilization.''; PubMed Europe PMC Scholia
  39. Sheftel AD, Stehling O, Pierik AJ, Netz DJ, Kerscher S, Elsässer HP, Wittig I, Balk J, Brandt U, Lill R.; ''Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I.''; PubMed Europe PMC Scholia
  40. Carilla-Latorre S, Gallardo ME, Annesley SJ, Calvo-Garrido J, Graña O, Accari SL, Smith PK, Valencia A, Garesse R, Fisher PR, Escalante R.; ''MidA is a putative methyltransferase that is required for mitochondrial complex I function.''; PubMed Europe PMC Scholia
  41. MacLennan DH, Lenaz G, Szarkowska L.; ''Studies on the mechanims of oxidative phosphorylation. IX. Effect of cytochrome c on energy-linked processes.''; PubMed Europe PMC Scholia
  42. Mitchell P.; ''Possible molecular mechanisms of the protonmotive function of cytochrome systems.''; PubMed Europe PMC Scholia
  43. Coates PM, Tanaka K.; ''Molecular basis of mitochondrial fatty acid oxidation defects.''; PubMed Europe PMC Scholia
  44. Echtay KS, Murphy MP, Smith RA, Talbot DA, Brand MD.; ''Superoxide activates mitochondrial uncoupling protein 2 from the matrix side. Studies using targeted antioxidants.''; PubMed Europe PMC Scholia
  45. Friedrich T, Böttcher B.; ''The gross structure of the respiratory complex I: a Lego System.''; PubMed Europe PMC Scholia
  46. Guzzo G, Sciacovelli M, Bernardi P, Rasola A.; ''Inhibition of succinate dehydrogenase by the mitochondrial chaperone TRAP1 has anti-oxidant and anti-apoptotic effects on tumor cells.''; PubMed Europe PMC Scholia

History

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CompareRevisionActionTimeUserComment
114966view16:49, 25 January 2021ReactomeTeamReactome version 75
113410view11:48, 2 November 2020ReactomeTeamReactome version 74
112612view15:59, 9 October 2020ReactomeTeamReactome version 73
101528view11:39, 1 November 2018ReactomeTeamreactome version 66
101063view21:21, 31 October 2018ReactomeTeamreactome version 65
100594view19:55, 31 October 2018ReactomeTeamreactome version 64
100143view16:40, 31 October 2018ReactomeTeamreactome version 63
99693view15:09, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99281view12:45, 31 October 2018ReactomeTeamreactome version 62
93907view13:44, 16 August 2017ReactomeTeamreactome version 61
93481view11:24, 9 August 2017ReactomeTeamreactome version 61
86578view09:21, 11 July 2016ReactomeTeamreactome version 56
83426view11:11, 18 November 2015ReactomeTeamVersion54
81630view13:10, 21 August 2015ReactomeTeamVersion53
77091view08:38, 17 July 2014ReactomeTeamFixed remaining interactions
76797view12:18, 16 July 2014ReactomeTeamFixed remaining interactions
76120view10:18, 11 June 2014ReactomeTeamRe-fixing comment source
75832view11:40, 10 June 2014ReactomeTeamReactome 48 Update
75192view09:40, 9 May 2014AnweshaFixing comment source for displaying WikiPathways description
74837view10:06, 30 April 2014ReactomeTeamReactome46
74431view07:10, 19 April 2014EgonwRelocated an InfoBox.
68937view17:34, 8 July 2013MaintBotUpdated to 2013 gpml schema
45047view19:09, 6 October 2011ThomasOntology Term : 'energy metabolic pathway' added !
42118view21:58, 4 March 2011MaintBotAutomatic update
39928view05:56, 21 January 2011MaintBotNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
ADP Metabolite16761 (ChEBI)
ATP Metabolite15422 (ChEBI)
ATPase

complex

ComplexREACT_4862 (Reactome)
ATPase-ADP and

Pi complex

ComplexREACT_2435 (Reactome)
ATPase-ATP

complex

ComplexREACT_3945 (Reactome)
Complex I -

NADH: Ubiquinone oxidoreductase

ComplexREACT_6533 (Reactome)
Cytochrome

c oxidase

ComplexREACT_6661 (Reactome)
Cytochrome c

(oxidized)

ComplexREACT_13953 (Reactome)
Cytochrome c

(reduced)

ComplexREACT_13858 (Reactome)
ETF ComplexREACT_6490 (Reactome)
ETF

(reduced)

ComplexREACT_6568 (Reactome)
ETFDH ProteinQ16134 (UniProt)
FA anion:UCP

dimer "head- out" complex

ComplexREACT_6437 (Reactome)
FA anion:UCP

dimer "head-in" complex

ComplexREACT_6679 (Reactome)
FAD Metabolite16238 (ChEBI)
FADH2 Metabolite17877 (ChEBI)
Fatty Acid

"head-in"

UnknownREACT_6592 (Reactome)
Fatty Acid

"head-out"

UnknownREACT_6554 (Reactome)
Fatty Acid

anion "head-out"

UnknownREACT_6652 (Reactome)
Fatty Acid anion

"head-in"

UnknownREACT_6375 (Reactome)
H+ Metabolite15378 (ChEBI)
H2O Metabolite15377 (ChEBI)
Long-chain

fatty acid

Unknown15904 (ChEBI)
NAD+ Metabolite15846 (ChEBI)
NADH Metabolite16908 (ChEBI)
Orthophosp

hate

Metabolite18367 (ChEBI)
Oxygen Metabolite15379 (ChEBI)
Purine

nucleotide

UnknownREACT_6384 (Reactome)
Succinate

dehydrogenase complex (oxidised)

ComplexREACT_3127 (Reactome)
Succinate

dehydrogenase complex (reduced)

ComplexREACT_6402 (Reactome)
UCP

dimer

ComplexREACT_6463 (Reactome)
Ubiquinol-

cytochrome c reductase

ComplexREACT_6527 (Reactome)
ubiquinol Metabolite17976 (ChEBI)
ubiquinone Metabolite16389 (ChEBI)

Annotated Interactions

No annotated interactions

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