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High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease.

<div class='caption-title'>Cryo-EM structure of respiratory complex I from Y. lipolytica.</div>
<div class='caption-title'>Q molecule in the access pathway.</div>
<div class='caption-title'>Lipid binding sites and unique protein lipid arrangement at the junction of the membrane and matrix arms.</div>
Parey K, Haapanen O, Sharma V, Köfeler H, Züllig T, Prinz S, Siegmund K, Wittig I, Mills DJ, Vonck J, Kühlbrandt W, Zickermann V
OpenAccess logo Sci Adv 5 eaax9484 (2019)
Related entries: 6rfr, 6rfs

Cited: 58 times
EuropePMC logo PMID: 31844670

Abstract

Respiratory complex I is a redox-driven proton pump, accounting for a large part of the electrochemical gradient that powers mitochondrial adenosine triphosphate synthesis. Complex I dysfunction is associated with severe human diseases. Assembly of the one-megadalton complex I in the inner mitochondrial membrane requires assembly factors and chaperones. We have determined the structure of complex I from the aerobic yeast Yarrowia lipolytica by electron cryo-microscopy at 3.2-Å resolution. A ubiquinone molecule was identified in the access path to the active site. The electron cryo-microscopy structure indicated an unusual lipid-protein arrangement at the junction of membrane and matrix arms that was confirmed by molecular simulations. The structure of a complex I mutant and an assembly intermediate provide detailed molecular insights into the cause of a hereditary complex I-linked disease and complex I assembly in the inner mitochondrial membrane.

Reviews - 6rfq mentioned but not cited (2)

  1. Accessory Subunits of the Matrix Arm of Mitochondrial Complex I with a Focus on Subunit NDUFS4 and Its Role in Complex I Function and Assembly. Kahlhöfer F, Gansen M, Zickermann V. Life (Basel) 11 455 (2021)
  2. The Mysterious Multitude: Structural Perspective on the Accessory Subunits of Respiratory Complex I. Padavannil A, Ayala-Hernandez MG, Castellanos-Silva EA, Letts JA. Front Mol Biosci 8 798353 (2021)

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  1. Gene Mining for Conserved, Non-Annotated Proteins of Podosphaera xanthii Identifies Novel Target Candidates for Controlling Powdery Mildews by Spray-Induced Gene Silencing. Ruiz-Jiménez L, Polonio Á, Vielba-Fernández A, Pérez-García A, Fernández-Ortuño D. J Fungi (Basel) 7 735 (2021)


Reviews citing this publication (13)

  1. Mitochondrial Metabolism as a Target for Cancer Therapy. Vasan K, Werner M, Chandel NS. Cell Metab 32 341-352 (2020)
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  10. Cryo-EM as a tool to study bacterial efflux systems and the membrane proteome. Klenotic PA, Morgan CE, Yu EW. Fac Rev 10 24 (2021)
  11. Group II Intron-Encoded Proteins (IEPs/Maturases) as Key Regulators of Nad1 Expression and Complex I Biogenesis in Land Plant Mitochondria. Mizrahi R, Shevtsov-Tal S, Ostersetzer-Biran O. Genes (Basel) 13 1137 (2022)
  12. Mitochondrial complex I as a therapeutic target for Alzheimer's disease. Trushina E, Trushin S, Hasan MF. Acta Pharm Sin B 12 483-495 (2022)
  13. The road to the structure of the mitochondrial respiratory chain supercomplex. Caruana NJ, Stroud DA. Biochem Soc Trans 48 621-629 (2020)

Articles citing this publication (42)

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  2. Mitochondrial complex I structure reveals ordered water molecules for catalysis and proton translocation. Grba DN, Hirst J. Nat Struct Mol Biol 27 892-900 (2020)
  3. Essential role of accessory subunit LYRM6 in the mechanism of mitochondrial complex I. Galemou Yoga E, Parey K, Djurabekova A, Haapanen O, Siegmund K, Zwicker K, Sharma V, Zickermann V, Angerer H. Nat Commun 11 6008 (2020)
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  5. High-resolution structure and dynamics of mitochondrial complex I-Insights into the proton pumping mechanism. Parey K, Lasham J, Mills DJ, Djurabekova A, Haapanen O, Yoga EG, Xie H, Kühlbrandt W, Sharma V, Vonck J, Zickermann V. Sci Adv 7 eabj3221 (2021)
  6. IACS-010759, a potent inhibitor of glycolysis-deficient hypoxic tumor cells, inhibits mitochondrial respiratory complex I through a unique mechanism. Tsuji A, Akao T, Masuya T, Murai M, Miyoshi H. J Biol Chem 295 7481-7491 (2020)
  7. Structural basis of mammalian respiratory complex I inhibition by medicinal biguanides. Bridges HR, Blaza JN, Yin Z, Chung I, Pollak MN, Hirst J. Science 379 351-357 (2023)
  8. Structure of the mature kinetoplastids mitoribosome and insights into its large subunit biogenesis. Soufari H, Waltz F, Parrot C, Durrieu-Gaillard S, Bochler A, Kuhn L, Sissler M, Hashem Y. Proc Natl Acad Sci U S A 117 29851-29861 (2020)
  9. Analysis of the assembly pathway for membrane subunits of Complex I reveals that subunit L (ND5) can assemble last in E. coli. Zhang F, Vik SB. BBA Adv 1 100027 (2021)
  10. Conformational changes in mitochondrial complex I of the thermophilic eukaryote Chaetomium thermophilum. Laube E, Meier-Credo J, Langer JD, Kühlbrandt W. Sci Adv 8 eadc9952 (2022)
  11. Cork-in-bottle mechanism of inhibitor binding to mammalian complex I. Chung I, Serreli R, Cross JB, Di Francesco ME, Marszalek JR, Hirst J. Sci Adv 7 eabg4000 (2021)
  12. Deactivation blocks proton pathways in the mitochondrial complex I. Röpke M, Riepl D, Saura P, Di Luca A, Mühlbauer ME, Jussupow A, Gamiz-Hernandez AP, Kaila VRI. Proc Natl Acad Sci U S A 118 e2019498118 (2021)
  13. Atomic structure of a mitochondrial complex I intermediate from vascular plants. Maldonado M, Padavannil A, Zhou L, Guo F, Letts JA. Elife 9 (2020)
  14. Cryo-EM structures define ubiquinone-10 binding to mitochondrial complex I and conformational transitions accompanying Q-site occupancy. Chung I, Wright JJ, Bridges HR, Ivanov BS, Biner O, Pereira CS, Arantes GM, Hirst J. Nat Commun 13 2758 (2022)
  15. Functional Water Wires Catalyze Long-Range Proton Pumping in the Mammalian Respiratory Complex I. Röpke M, Saura P, Riepl D, Pöverlein MC, Kaila VRI. J Am Chem Soc 142 21758-21766 (2020)
  16. Resting mitochondrial complex I from Drosophila melanogaster adopts a helix-locked state. Padavannil A, Murari A, Rhooms SK, Owusu-Ansah E, Letts JA. Elife 12 e84415 (2023)
  17. Structure of inhibitor-bound mammalian complex I. Bridges HR, Fedor JG, Blaza JN, Di Luca A, Jussupow A, Jarman OD, Wright JJ, Agip AA, Gamiz-Hernandez AP, Roessler MM, Kaila VRI, Hirst J. Nat Commun 11 5261 (2020)
  18. A Quinol Anion as Catalytic Intermediate Coupling Proton Translocation With Electron Transfer in E. coli Respiratory Complex I. Nuber F, Mérono L, Oppermann S, Schimpf J, Wohlwend D, Friedrich T. Front Chem 9 672969 (2021)
  19. Cryo-EM structures of mitochondrial respiratory complex I from Drosophila melanogaster. Agip AA, Chung I, Sanchez-Martinez A, Whitworth AJ, Hirst J. Elife 12 e84424 (2023)
  20. FTSH PROTEASE 3 facilitates Complex I degradation through a direct interaction with the Complex I subunit PSST. Ghifari AS, Ivanova A, Berkowitz O, Whelan J, Murcha MW. Plant Cell 35 3092-3108 (2023)
  21. Key role of quinone in the mechanism of respiratory complex I. Gutiérrez-Fernández J, Kaszuba K, Minhas GS, Baradaran R, Tambalo M, Gallagher DT, Sazanov LA. Nat Commun 11 4135 (2020)
  22. Oversized ubiquinones as molecular probes for structural dynamics of the ubiquinone reaction site in mitochondrial respiratory complex I. Uno S, Masuya T, Shinzawa-Itoh K, Lasham J, Haapanen O, Shiba T, Inaoka DK, Sharma V, Murai M, Miyoshi H. J Biol Chem 295 2449-2463 (2020)
  23. Paracoccus denitrificans: a genetically tractable model system for studying respiratory complex I. Jarman OD, Biner O, Wright JJ, Hirst J. Sci Rep 11 10143 (2021)
  24. Quinone Catalysis Modulates Proton Transfer Reactions in the Membrane Domain of Respiratory Complex I. Kim H, Saura P, Pöverlein MC, Gamiz-Hernandez AP, Kaila VRI. J Am Chem Soc 145 17075-17086 (2023)
  25. Subunits E-F-G of E. coli Complex I can form an active complex when expressed alone, but in time-delayed assembly co-expression of B-CD-E-F-G is optimal. Alkhaldi HA, Vik SB. Biochim Biophys Acta Bioenerg 1863 148593 (2022)
  26. A conserved arginine residue is critical for stabilizing the N2 FeS cluster in mitochondrial complex I. Hameedi MA, Grba DN, Richardson KH, Jones AJY, Song W, Roessler MM, Wright JJ, Hirst J. J Biol Chem 100474 (2021)
  27. Assembly and phylogeographical analysis of novel Taenia solium mitochondrial genomes suggest stratification within the African-American genotype. Jiménez-Avalos G, Soto-Obando A, Solis M, Gilman RH, Cama V, Gonzalez AE, García HH, Sheen P, Requena D, Zimic M, Cysticercosis Working Group in Peru. Parasit Vectors 16 349 (2023)
  28. Biochemical consequences of two clinically relevant ND-gene mutations in Escherichia coli respiratory complex I. Nuber F, Schimpf J, di Rago JP, Tribouillard-Tanvier D, Procaccio V, Martin-Negrier ML, Trimouille A, Biner O, von Ballmoos C, Friedrich T. Sci Rep 11 12641 (2021)
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  30. Horizontal proton transfer across the antiporter-like subunits in mitochondrial respiratory complex I. Zdorevskyi O, Djurabekova A, Lasham J, Sharma V. Chem Sci 14 6309-6318 (2023)
  31. Insights into complex I assembly: Function of NDUFAF1 and a link with cardiolipin remodeling. Schiller J, Laube E, Wittig I, Kühlbrandt W, Vonck J, Zickermann V. Sci Adv 8 eadd3855 (2022)
  32. Mechanism of rotenone binding to respiratory complex I depends on ligand flexibility. Pereira CS, Teixeira MH, Russell DA, Hirst J, Arantes GM. Sci Rep 13 6738 (2023)
  33. Mutation at the entrance of the quinone cavity severely disrupts quinone binding in respiratory complex I. Yi JT, Wang P, Stuchebrukhov AA. Sci Rep 13 20413 (2023)
  34. Oxygen-Sensitive Metalloprotein Structure Determination by Cryo-Electron Microscopy. Cherrier MV, Vernède X, Fenel D, Martin L, Arragain B, Neumann E, Fontecilla-Camps JC, Schoehn G, Nicolet Y. Biomolecules 12 441 (2022)
  35. Phospholipids can regulate complex I assembly independent of their role in maintaining mitochondrial membrane integrity. Murari A, Rhooms SK, Vimal D, Hossain KFB, Saini S, Villanueva M, Schlame M, Owusu-Ansah E. Cell Rep 42 112846 (2023)
  36. Protocol for the Analysis of Yeast and Human Mitochondrial Respiratory Chain Complexes and Supercomplexes by Blue Native Electrophoresis. Timón-Gómez A, Pérez-Pérez R, Nyvltova E, Ugalde C, Fontanesi F, Barrientos A. STAR Protoc 1 (2020)
  37. Quinone binding in respiratory complex I: Going through the eye of a needle. The squeeze-in mechanism of passing the narrow entrance of the quinone site. Dhananjayan N, Wang P, Leontyev I, Stuchebrukhov AA. Photochem Photobiol Sci 21 1-12 (2022)
  38. Single protonation of the reduced quinone in respiratory complex I drives four-proton pumping. Stuchebrukhov AA, Hayashi T. FEBS Lett 597 237-245 (2023)
  39. Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4-/- mouse. Yin Z, Agip AA, Bridges HR, Hirst J. EMBO J 43 225-249 (2024)
  40. Structures of Tetrahymena's respiratory chain reveal the diversity of eukaryotic core metabolism. Zhou L, Maldonado M, Padavannil A, Guo F, Letts JA. Science 376 831-839 (2022)
  41. The Discovery and Development of Thienopyrimidines as Inhibitors of Helicobacter pylori That Act through Inhibition of the Respiratory Complex I. Mugengana AK, Vita NA, Brown Gandt A, Moran K, Agyapong G, Sharma LK, Griffith EC, Liu J, Yang L, Gavrish E, Hevener KE, LaFleur MD, Lee RE. ACS Infect Dis 7 1044-1058 (2021)
  42. The coupling mechanism of mammalian mitochondrial complex I. Gu J, Liu T, Guo R, Zhang L, Yang M. Nat Struct Mol Biol 29 172-182 (2022)


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