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Title | High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease. |
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Journal, issue, pages | Sci Adv, Vol. 5, Issue 12, Page eaax9484, Year 2019 |
Publish date | Dec 11, 2019 |
![]() | Kristian Parey / Outi Haapanen / Vivek Sharma / Harald Köfeler / Thomas Züllig / Simone Prinz / Karin Siegmund / Ilka Wittig / Deryck J Mills / Janet Vonck / Werner Kühlbrandt / Volker Zickermann / ![]() ![]() ![]() |
PubMed 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 ...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 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. |
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Methods | EM (single particle) |
Resolution | 3.2 - 4.04 Å |
Structure data | EMDB-4872, PDB-6rfq: |
Chemicals | ![]() ChemComp-SF4: ![]() ChemComp-FES: ![]() ChemComp-FMN: ![]() ChemComp-NDP: ![]() ChemComp-3PE: ![]() ChemComp-LMN: ![]() ChemComp-CDL: ![]() ChemComp-ZMP: ![]() ChemComp-PLC: ![]() ChemComp-T7X: ![]() ChemComp-CPL: ![]() ChemComp-ZN: ![]() ChemComp-UQ9: |
Source |
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![]() | OXIDOREDUCTASE / Complex I / NADH dehydrogenase / Mitochondrion Proton pumping / Ubiquinone |