6g72 › Citations

Cryo-EM structures of complex I from mouse heart mitochondria in two biochemically defined states.

Agip AA, Blaza JN, Bridges HR, Viscomi C, Rawson S, Muench SP, Hirst J
Nat Struct Mol Biol 25 548-556 (2018)
Cited: 128 times
EuropePMC logo PMID: 29915388

Abstract

Complex I (NADH:ubiquinone oxidoreductase) uses the reducing potential of NADH to drive protons across the energy-transducing inner membrane and power oxidative phosphorylation in mammalian mitochondria. Recent cryo-EM analyses have produced near-complete models of all 45 subunits in the bovine, ovine and porcine complexes and have identified two states relevant to complex I in ischemia-reperfusion injury. Here, we describe the 3.3-Å structure of complex I from mouse heart mitochondria, a biomedically relevant model system, in the 'active' state. We reveal a nucleotide bound in subunit NDUFA10, a nucleoside kinase homolog, and define mechanistically critical elements in the mammalian enzyme. By comparisons with a 3.9-Å structure of the 'deactive' state and with known bacterial structures, we identify differences in helical geometry in the membrane domain that occur upon activation or that alter the positions of catalytically important charged residues. Our results demonstrate the capability of cryo-EM analyses to challenge and develop mechanistic models for mammalian complex I.

Reviews - 6g72 mentioned but not cited (1)

  1. Analysis of Human Mutations in the Supernumerary Subunits of Complex I. Dang QL, Phan DH, Johnson AN, Pasapuleti M, Alkhaldi HA, Zhang F, Vik SB. Life (Basel) 10 E296 (2020)

Articles - 6g72 mentioned but not cited (10)

  1. Cryo-EM structures of complex I from mouse heart mitochondria in two biochemically defined states. Agip AA, Blaza JN, Bridges HR, Viscomi C, Rawson S, Muench SP, Hirst J. Nat Struct Mol Biol 25 548-556 (2018)
  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. ND3 Cys39 in complex I is exposed during mitochondrial respiration. Burger N, James AM, Mulvey JF, Hoogewijs K, Ding S, Fearnley IM, Loureiro-López M, Norman AAI, Arndt S, Mottahedin A, Sauchanka O, Hartley RC, Krieg T, Murphy MP. Cell Chem Biol 29 636-649.e14 (2022)
  4. 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)
  5. 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)
  6. Plant-specific features of respiratory supercomplex I + III2 from Vigna radiata. Maldonado M, Fan Z, Abe KM, Letts JA. Nat Plants 9 157-168 (2023)
  7. 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)
  8. Antibacterial activity and antibacterial mechanism of flavaspidic acid BB against Staphylococcus haemelyticus. Liu J, Liu R, Deng R, Zheng S, Shen Z. BMC Microbiol 23 276 (2023)
  9. Case Reports Case report: A rare variant m.4135T>C in the MT-ND1 gene leads to Leber hereditary optic neuropathy and altered respiratory chain supercomplexes. Rákosníková T, Kelifová S, Štufková H, Lokvencová K, Lišková P, Kousal B, Honzík T, Hansíková H, Martínek V, Tesařová M. Front Genet 14 1182288 (2023)
  10. 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)


Reviews citing this publication (26)

  1. Generation of Reactive Oxygen Species by Mitochondria. Hernansanz-Agustín P, Enríquez JA. Antioxidants (Basel) 10 415 (2021)
  2. Mammalian Respiratory Complex I Through the Lens of Cryo-EM. Agip AA, Blaza JN, Fedor JG, Hirst J. Annu Rev Biophys 48 165-184 (2019)
  3. Frontiers in Cryo Electron Microscopy of Complex Macromolecular Assemblies. Ognjenović J, Grisshammer R, Subramaniam S. Annu Rev Biomed Eng 21 395-415 (2019)
  4. Structure of respiratory complex I - An emerging blueprint for the mechanism. Kampjut D, Sazanov LA. Curr Opin Struct Biol 74 102350 (2022)
  5. The assembly, regulation and function of the mitochondrial respiratory chain. Vercellino I, Sazanov LA. Nat Rev Mol Cell Biol 23 141-161 (2022)
  6. Complexome Profiling: Assembly and Remodeling of Protein Complexes. Wittig I, Malacarne PF. Int J Mol Sci 22 7809 (2021)
  7. 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)
  8. Preventing mitochondrial reverse electron transport as a strategy for cardioprotection. Prag HA, Murphy MP, Krieg T. Basic Res Cardiol 118 34 (2023)
  9. Ubiquinone Binding and Reduction by Complex I-Open Questions and Mechanistic Implications. Galemou Yoga E, Schiller J, Zickermann V. Front Chem 9 672851 (2021)
  10. Evolution of complex I-like respiratory complexes. Yu H, Schut GJ, Haja DK, Adams MWW, Li H. J Biol Chem 296 100740 (2021)
  11. Insights from Drosophila on mitochondrial complex I. Rhooms SK, Murari A, Goparaju NSV, Vilanueva M, Owusu-Ansah E. Cell Mol Life Sci 77 607-618 (2020)
  12. Making the leap from structure to mechanism: are the open states of mammalian complex I identified by cryoEM resting states or catalytic intermediates? Chung I, Grba DN, Wright JJ, Hirst J. Curr Opin Struct Biol 77 102447 (2022)
  13. Organization of the Respiratory Supercomplexes in Cells with Defective Complex III: Structural Features and Metabolic Consequences. Rugolo M, Zanna C, Ghelli AM. Life (Basel) 11 351 (2021)
  14. Tackling Dysfunction of Mitochondrial Bioenergetics in the Brain. Zanfardino P, Doccini S, Santorelli FM, Petruzzella V. Int J Mol Sci 22 8325 (2021)
  15. The biogenesis and regulation of the plant oxidative phosphorylation system. Ghifari AS, Saha S, Murcha MW. Plant Physiol 192 728-747 (2023)
  16. The functional significance of mitochondrial respiratory chain supercomplexes. Kohler A, Barrientos A, Fontanesi F, Ott M. EMBO Rep 24 e57092 (2023)
  17. A primordial target: Mitochondria mediate both primary and collateral anesthetic effects of volatile anesthetics. Perouansky M, Johnson-Schlitz D, Sedensky MM, Morgan PG. Exp Biol Med (Maywood) 248 545-552 (2023)
  18. Detergents and alternatives in cryo-EM studies of membrane proteins. Li S. Acta Biochim Biophys Sin (Shanghai) 54 1049-1056 (2022)
  19. Electron microscopy of cardiac 3D nanodynamics: form, function, future. Kohl P, Greiner J, Rog-Zielinska EA. Nat Rev Cardiol (2022)
  20. From the 'black box' to 'domino effect' mechanism: what have we learned from the structures of respiratory complex I. Sazanov LA. Biochem J 480 319-333 (2023)
  21. Membranes under the Magnetic Lens: A Dive into the Diverse World of Membrane Protein Structures Using Cryo-EM. Piper SJ, Johnson RM, Wootten D, Sexton PM. Chem Rev 122 13989-14017 (2022)
  22. Monolysocardiolipin (MLCL) interactions with mitochondrial membrane proteins. Duncan AL. Biochem Soc Trans 48 993-1004 (2020)
  23. Research journey of respirasome. Wu M, Gu J, Zong S, Guo R, Liu T, Yang M. Protein Cell 11 318-338 (2020)
  24. Systems Biochemistry Approaches to Defining Mitochondrial Protein Function. Sung AY, Floyd BJ, Pagliarini DJ. Cell Metab 31 669-678 (2020)
  25. 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)
  26. 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 (91)

  1. Specific features and assembly of the plant mitochondrial complex I revealed by cryo-EM. Soufari H, Parrot C, Kuhn L, Waltz F, Hashem Y. Nat Commun 11 5195 (2020)
  2. Endoplasmic reticulum stress-induced complex I defect: Central role of calcium overload. Mohsin AA, Thompson J, Hu Y, Hollander J, Lesnefsky EJ, Chen Q. Arch Biochem Biophys 683 108299 (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)
  4. Respiratory complex and tissue lineage drive recurrent mutations in tumour mtDNA. Gorelick AN, Kim M, Chatila WK, La K, Hakimi AA, Berger MF, Taylor BS, Gammage PA, Reznik E. Nat Metab 3 558-570 (2021)
  5. Impaired complex I repair causes recessive Leber's hereditary optic neuropathy. Stenton SL, Sheremet NL, Catarino CB, Andreeva NA, Assouline Z, Barboni P, Barel O, Berutti R, Bychkov I, Caporali L, Capristo M, Carbonelli M, Cascavilla ML, Charbel Issa P, Freisinger P, Gerber S, Ghezzi D, Graf E, Heidler J, Hempel M, Heon E, Itkis YS, Javasky E, Kaplan J, Kopajtich R, Kornblum C, Kovacs-Nagy R, Krylova TD, Kunz WS, La Morgia C, Lamperti C, Ludwig C, Malacarne PF, Maresca A, Mayr JA, Meisterknecht J, Nevinitsyna TA, Palombo F, Pode-Shakked B, Shmelkova MS, Strom TM, Tagliavini F, Tzadok M, van der Ven AT, Vignal-Clermont C, Wagner M, Zakharova EY, Zhorzholadze NV, Rozet JM, Carelli V, Tsygankova PG, Klopstock T, Wittig I, Prokisch H. J Clin Invest 131 138267 (2021)
  6. Water-Gated Proton Transfer Dynamics in Respiratory Complex I. Mühlbauer ME, Saura P, Nuber F, Di Luca A, Friedrich T, Kaila VRI. J Am Chem Soc 142 13718-13728 (2020)
  7. A ferredoxin bridge connects the two arms of plant mitochondrial complex I. Klusch N, Senkler J, Yildiz Ö, Kühlbrandt W, Braun HP. Plant Cell 33 2072-2091 (2021)
  8. Structure of the respiratory MBS complex reveals iron-sulfur cluster catalyzed sulfane sulfur reduction in ancient life. Yu H, Haja DK, Schut GJ, Wu CH, Meng X, Zhao G, Li H, Adams MWW. Nat Commun 11 5953 (2020)
  9. 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)
  10. Mild depolarization of the inner mitochondrial membrane is a crucial component of an anti-aging program. Vyssokikh MY, Holtze S, Averina OA, Lyamzaev KG, Panteleeva AA, Marey MV, Zinovkin RA, Severin FF, Skulachev MV, Fasel N, Hildebrandt TB, Skulachev VP. Proc Natl Acad Sci U S A 117 6491-6501 (2020)
  11. Redox-coupled proton pumping drives carbon concentration in the photosynthetic complex I. Schuller JM, Saura P, Thiemann J, Schuller SK, Gamiz-Hernandez AP, Kurisu G, Nowaczyk MM, Kaila VRI. Nat Commun 11 494 (2020)
  12. Defining the mechanism of action of S1QELs, specific suppressors of superoxide production in the quinone-reaction site in mitochondrial complex I. Banba A, Tsuji A, Kimura H, Murai M, Miyoshi H. J Biol Chem 294 6550-6561 (2019)
  13. 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)
  14. An antibody toolbox to track complex I assembly defines AIF's mitochondrial function. Murari A, Rhooms SK, Goparaju NS, Villanueva M, Owusu-Ansah E. J Cell Biol 219 e202001071 (2020)
  15. Multiscale Simulations of Biological Membranes: The Challenge To Understand Biological Phenomena in a Living Substance. Enkavi G, Javanainen M, Kulig W, Róg T, Vattulainen I. Chem. Rev. 119 5607-5774 (2019)
  16. 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)
  17. Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I. Alahmad A, Nasca A, Heidler J, Thompson K, Oláhová M, Legati A, Lamantea E, Meisterknecht J, Spagnolo M, He L, Alameer S, Hakami F, Almehdar A, Ardissone A, Alston CL, McFarland R, Wittig I, Ghezzi D, Taylor RW. EMBO Mol Med 12 e12619 (2020)
  18. 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)
  19. Gene fragmentation and RNA editing without borders: eccentric mitochondrial genomes of diplonemids. Kaur B, Záhonová K, Valach M, Faktorová D, Prokopchuk G, Burger G, Lukeš J. Nucleic Acids Res 48 2694-2708 (2020)
  20. Atomic structure of a mitochondrial complex I intermediate from vascular plants. Maldonado M, Padavannil A, Zhou L, Guo F, Letts JA. Elife 9 (2020)
  21. Cryo-EM structure of respiratory complex I at work. Parey K, Brandt U, Xie H, Mills DJ, Siegmund K, Vonck J, Kühlbrandt W, Zickermann V. Elife 7 (2018)
  22. 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)
  23. Hydroxylated Rotenoids Selectively Inhibit the Proliferation of Prostate Cancer Cells. Russell DA, Bridges HR, Serreli R, Kidd SL, Mateu N, Osberger TJ, Sore HF, Hirst J, Spring DR. J Nat Prod 83 1829-1845 (2020)
  24. IDH2-mediated regulation of the biogenesis of the oxidative phosphorylation system. Murari A, Goparaju NSV, Rhooms SK, Hossain KFB, Liang FG, Garcia CJ, Osei C, Liu T, Li H, Kitsis RN, Patel R, Owusu-Ansah E. Sci Adv 8 eabl8716 (2022)
  25. Multi-mtDNA Variants May Be a Factor Contributing to Mitochondrial Function Variety in the Skin-Derived Fibroblasts of Leber's Hereditary Optic Neuropathy Patients. Yao S, Zhou Q, Yang M, Li Y, Jin X, Guo Q, Yang L, Qin F, Lei B. Front Mol Neurosci 15 920221 (2022)
  26. NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate. D'Angelo L, Astro E, De Luise M, Kurelac I, Umesh-Ganesh N, Ding S, Fearnley IM, Gasparre G, Zeviani M, Porcelli AM, Fernandez-Vizarra E, Iommarini L. Cell Rep 35 109002 (2021)
  27. Spatial arrangement of proteins in planar and curved membranes by PPM 3.0. Lomize AL, Todd SC, Pogozheva ID. Protein Sci 31 209-220 (2022)
  28. 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 296 100474 (2021)
  29. AIFM1 is a component of the mitochondrial disulfide relay that drives complex I assembly through efficient import of NDUFS5. Salscheider SL, Gerlich S, Cabrera-Orefice A, Peker E, Rothemann RA, Murschall LM, Finger Y, Szczepanowska K, Ahmadi ZA, Guerrero-Castillo S, Erdogan A, Becker M, Ali M, Habich M, Petrungaro C, Burdina N, Schwarz G, Klußmann M, Neundorf I, Stroud DA, Ryan MT, Trifunovic A, Brandt U, Riemer J. EMBO J 41 e110784 (2022)
  30. Chlamydomonas reinhardtii as a plant model system to study mitochondrial complex I dysfunction. Subrahmanian N, Castonguay AD, Fatnes TA, Hamel PP. Plant Direct 4 e00200 (2020)
  31. Complex I mutations synergize to worsen the phenotypic expression of Leber's hereditary optic neuropathy. Ji Y, Zhang J, Lu Y, Yi Q, Chen M, Xie S, Mao X, Xiao Y, Meng F, Zhang M, Yang R, Guan MX. J Biol Chem 295 13224-13238 (2020)
  32. 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)
  33. Cryo-electron microscopy reveals how acetogenins inhibit mitochondrial respiratory complex I. Grba DN, Blaza JN, Bridges HR, Agip AA, Yin Z, Murai M, Miyoshi H, Hirst J. J Biol Chem 298 101602 (2022)
  34. Exploring the quinone/inhibitor-binding pocket in mitochondrial respiratory complex I by chemical biology approaches. Uno S, Kimura H, Murai M, Miyoshi H. J. Biol. Chem. 294 679-696 (2019)
  35. 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)
  36. Leveraging the Entirety of the Protein Data Bank to Enable Improved Structure Prediction Based on Cross-Link Data. Keller A, Chavez JD, Tang X, Bruce JE. J Proteome Res 20 1087-1095 (2021)
  37. Locking loop movement in the ubiquinone pocket of complex I disengages the proton pumps. Cabrera-Orefice A, Yoga EG, Wirth C, Siegmund K, Zwicker K, Guerrero-Castillo S, Zickermann V, Hunte C, Brandt U. Nat Commun 9 4500 (2018)
  38. Long-lived mitochondrial cristae proteins in mouse heart and brain. Bomba-Warczak E, Edassery SL, Hark TJ, Savas JN. J Cell Biol 220 e202005193 (2021)
  39. Membrane-domain mutations in respiratory complex I impede catalysis but do not uncouple proton pumping from ubiquinone reduction. Jarman OD, Hirst J. PNAS Nexus 1 pgac276 (2022)
  40. 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)
  41. 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)
  42. 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)
  43. Resolving Chemical Dynamics in Biological Energy Conversion: Long-Range Proton-Coupled Electron Transfer in Respiratory Complex I. Kaila VRI. Acc Chem Res 54 4462-4473 (2021)
  44. Respiratory chain Complex I of unparalleled divergence in diplonemids. Valach M, Léveillé-Kunst A, Gray MW, Burger G. J. Biol. Chem. 293 16043-16056 (2018)
  45. Respiratory complex I with charge symmetry in the membrane arm pumps protons. Hoeser F, Tausend H, Götz S, Wohlwend D, Einsle O, Günther S, Friedrich T. Proc Natl Acad Sci U S A 119 e2123090119 (2022)
  46. Structural insights into NDH-1 mediated cyclic electron transfer. Zhang C, Shuai J, Ran Z, Zhao J, Wu Z, Liao R, Wu J, Ma W, Lei M. Nat Commun 11 888 (2020)
  47. Structure of Escherichia coli respiratory complex I reconstituted into lipid nanodiscs reveals an uncoupled conformation. Kolata P, Efremov RG. Elife 10 e68710 (2021)
  48. Structure of mycobacterial respiratory complex I. Liang Y, Plourde A, Bueler SA, Liu J, Brzezinski P, Vahidi S, Rubinstein JL. Proc Natl Acad Sci U S A 120 e2214949120 (2023)
  49. 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)
  50. 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)
  51. cAMP/PKA Signaling Modulates Mitochondrial Supercomplex Organization. Signorile A, Pacelli C, Palese LL, Santeramo A, Roca E, Cocco T, De Rasmo D. Int J Mol Sci 23 9655 (2022)
  52. A lethal mitonuclear incompatibility in complex I of natural hybrids. Moran BM, Payne CY, Powell DL, Iverson ENK, Donny AE, Banerjee SM, Langdon QK, Gunn TR, Rodriguez-Soto RA, Madero A, Baczenas JJ, Kleczko KM, Liu F, Matney R, Singhal K, Leib RD, Hernandez-Perez O, Corbett-Detig R, Frydman J, Gifford C, Schartl M, Havird JC, Schumer M. Nature (2024)
  53. A universal coupling mechanism of respiratory complex I. Kravchuk V, Petrova O, Kampjut D, Wojciechowska-Bason A, Breese Z, Sazanov L. Nature 609 808-814 (2022)
  54. An Exploration of the Coherent Effects between METTL3 and NDUFA10 on Alzheimer's Disease. Yang L, Pang X, Guo W, Zhu C, Yu L, Song X, Wang K, Pang C. Int J Mol Sci 24 10111 (2023)
  55. Analysis of Human Clinical Mutations of Mitochondrial ND1 in a Bacterial Model System for Complex I. Alkhaldi HA, Phan DH, Vik SB. Life (Basel) 12 1934 (2022)
  56. Analysis of compound heterozygous and homozygous mutations found in peripheral subunits of human respiratory Complex I, NDUFS1, NDUFS2, NDUFS8 and NDUFV1, by modeling in the E. coli enzyme. Alkhaldi HA, Vik SB. Mitochondrion 68 87-104 (2023)
  57. Assocation Between Leber's Hereditary Optic Neuropathy and MT-ND1 3460G>A Mutation-Induced Alterations in Mitochondrial Function, Apoptosis, and Mitophagy. Zhang J, Ji Y, Chen J, Xu M, Wang G, Ci X, Lin B, Mo JQ, Zhou X, Guan MX. Invest Ophthalmol Vis Sci 62 38 (2021)
  58. Atomic structures of respiratory complex III2, complex IV, and supercomplex III2-IV from vascular plants. Maldonado M, Guo F, Letts JA. Elife 10 (2021)
  59. Dissecting the concordant and disparate roles of NDUFAF3 and NDUFAF4 in mitochondrial complex I biogenesis. Murari A, Rhooms SK, Garcia C, Liu T, Li H, Mishra B, Deshong C, Owusu-Ansah E. iScience 24 102869 (2021)
  60. Energetics and Dynamics of Proton-Coupled Electron Transfer in the NADH/FMN Site of Respiratory Complex I. Saura P, Kaila VRI. J. Am. Chem. Soc. 141 5710-5719 (2019)
  61. Forty years in cryoEM of membrane proteins. Kühlbrandt W. Microscopy (Oxf) 71 i30-i50 (2022)
  62. Functional Dynamics of an Ancient Membrane-Bound Hydrogenase. Mühlbauer ME, Gamiz-Hernandez AP, Kaila VRI. J Am Chem Soc 143 20873-20883 (2021)
  63. 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)
  64. High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease. Parey K, Haapanen O, Sharma V, Köfeler H, Züllig T, Prinz S, Siegmund K, Wittig I, Mills DJ, Vonck J, Kühlbrandt W, Zickermann V. Sci Adv 5 eaax9484 (2019)
  65. How cardiolipin modulates the dynamics of respiratory complex I. Jussupow A, Di Luca A, Kaila VRI. Sci Adv 5 eaav1850 (2019)
  66. Human clinical mutations in mitochondrially encoded subunits of Complex I can be successfully modeled in E. coli. Zhang F, Dang QL, Vik SB. Mitochondrion 64 59-72 (2022)
  67. Human frataxin, the Friedreich ataxia deficient protein, interacts with mitochondrial respiratory chain. Doni D, Cavion F, Bortolus M, Baschiera E, Muccioli S, Tombesi G, d'Ettorre F, Ottaviani D, Marchesan E, Leanza L, Greggio E, Ziviani E, Russo A, Bellin M, Sartori G, Carbonera D, Salviati L, Costantini P. Cell Death Dis 14 805 (2023)
  68. In vivo mitochondrial base editing via adeno-associated viral delivery to mouse post-mitotic tissue. Silva-Pinheiro P, Nash PA, Van Haute L, Mutti CD, Turner K, Minczuk M. Nat Commun 13 750 (2022)
  69. In vivo protein turnover rates in varying oxygen tensions nominate MYBBP1A as a mediator of the hyperoxia response. Chen X, Haribowo AG, Baik AH, Fossati A, Stevenson E, Chen YR, Reyes NS, Peng T, Matthay MA, Traglia M, Pico AR, Jarosz DF, Buchwalter A, Ghaemmaghami S, Swaney DL, Jain IH. Sci Adv 9 eadj4884 (2023)
  70. 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)
  71. Investigation of hydrated channels and proton pathways in a high-resolution cryo-EM structure of mammalian complex I. Grba DN, Chung I, Bridges HR, Agip AA, Hirst J. Sci Adv 9 eadi1359 (2023)
  72. Ion transfer mechanisms in Mrp-type antiporters from high resolution cryoEM and molecular dynamics simulations. Lee Y, Haapanen O, Altmeyer A, Kühlbrandt W, Sharma V, Zickermann V. Nat Commun 13 6091 (2022)
  73. 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)
  74. Knockdown of NDUFC1 inhibits cell proliferation, migration, and invasion of hepatocellular carcinoma. Han F, Liu J, Chu H, Cao D, Wu J, Fu H, Guo A, Chen W, Xu Y, Cheng X, Zhang Y. Front Oncol 12 860084 (2022)
  75. 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)
  76. Most mitochondrial dGTP is tightly bound to respiratory complex I through the NDUFA10 subunit. Molina-Granada D, González-Vioque E, Dibley MG, Cabrera-Pérez R, Vallbona-Garcia A, Torres-Torronteras J, Sazanov LA, Ryan MT, Cámara Y, Martí R. Commun Biol 5 620 (2022)
  77. 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)
  78. 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)
  79. Redox-dependent loss of flavin by mitochondria complex I is different in brain and heart. Yoval-Sánchez B, Ansari F, James J, Niatsetskaya Z, Sosunov S, Filipenko P, Tikhonova IG, Ten V, Wittig I, Rafikov R, Galkin A. Redox Biol 51 102258 (2022)
  80. Respiratory complex I in mitochondrial membrane catalyzes oversized ubiquinones. Ikunishi R, Otani R, Masuya T, Shinzawa-Itoh K, Shiba T, Murai M, Miyoshi H. J Biol Chem 299 105001 (2023)
  81. Reverse Electron Transfer by Respiratory Complex I Catalyzed in a Modular Proteoliposome System. Wright JJ, Biner O, Chung I, Burger N, Bridges HR, Hirst J. J Am Chem Soc 144 6791-6801 (2022)
  82. Role of Second Quinone Binding Site in Proton Pumping by Respiratory Complex I. Haapanen O, Djurabekova A, Sharma V. Front Chem 7 221 (2019)
  83. Structural basis for a complex I mutation that blocks pathological ROS production. Yin Z, Burger N, Kula-Alwar D, Aksentijević D, Bridges HR, Prag HA, Grba DN, Viscomi C, James AM, Mottahedin A, Krieg T, Murphy MP, Hirst J. Nat Commun 12 707 (2021)
  84. 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)
  85. Structure and mechanism of the Mrp complex, an ancient cation/proton antiporter. Steiner J, Sazanov L. Elife 9 (2020)
  86. Structure of the membrane-bound formate hydrogenlyase complex from Escherichia coli. Steinhilper R, Höff G, Heider J, Murphy BJ. Nat Commun 13 5395 (2022)
  87. 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)
  88. Structures of Respiratory Supercomplex I+III2 Reveal Functional and Conformational Crosstalk. Letts JA, Fiedorczuk K, Degliesposti G, Skehel M, Sazanov LA. Mol. Cell 75 1131-1146.e6 (2019)
  89. 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)
  90. The membrane domain of respiratory complex I accumulates during muscle aging in Drosophila melanogaster. Hossain KFB, Murari A, Mishra B, Owusu-Ansah E. Sci Rep 12 22433 (2022)
  91. Using light scattering to assess how phospholipid-protein interactions affect complex I functionality in liposomes. Eisermann J, Wright JJ, Wilton-Ely JDET, Hirst J, Roessler MM. RSC Chem Biol 4 386-398 (2023)


Quick links