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Yorodumi- PDB-6rfs: Cryo-EM structure of a respiratory complex I mutant lacking NDUFS4 -
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Open data
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Basic information
| Entry | Database: PDB / ID: 6rfs | ||||||
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| Title | Cryo-EM structure of a respiratory complex I mutant lacking NDUFS4 | ||||||
Components |
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Keywords | OXIDOREDUCTASE / Complex I / NADH dehydrogenase / Mitochondrion Proton pumping / Ubiquinone | ||||||
| Function / homology | Function and homology informationlipoate biosynthetic process / NADH dehydrogenase / TIM23 mitochondrial import inner membrane translocase complex / membrane protein complex / NADH dehydrogenase complex / ubiquinone biosynthetic process / mitochondrial [2Fe-2S] assembly complex / oxidoreductase activity, acting on NAD(P)H / NADH dehydrogenase activity / iron-sulfur cluster assembly ...lipoate biosynthetic process / NADH dehydrogenase / TIM23 mitochondrial import inner membrane translocase complex / membrane protein complex / NADH dehydrogenase complex / ubiquinone biosynthetic process / mitochondrial [2Fe-2S] assembly complex / oxidoreductase activity, acting on NAD(P)H / NADH dehydrogenase activity / iron-sulfur cluster assembly / acyl binding / ubiquinone binding / electron transport coupled proton transport / protein import into mitochondrial matrix / acyl carrier activity / NADH:ubiquinone reductase (H+-translocating) / mitochondrial respiratory chain complex I assembly / mitochondrial electron transport, NADH to ubiquinone / respiratory chain complex I / catalytic complex / NADH dehydrogenase (ubiquinone) activity / quinone binding / ATP synthesis coupled electron transport / aerobic respiration / respiratory electron transport chain / electron transport chain / mitochondrial membrane / mitochondrial intermembrane space / 2 iron, 2 sulfur cluster binding / NAD binding / FMN binding / 4 iron, 4 sulfur cluster binding / response to oxidative stress / oxidoreductase activity / mitochondrial inner membrane / protein-containing complex binding / mitochondrion / metal ion binding / membrane Similarity search - Function | ||||||
| Biological species | Yarrowia lipolytica (yeast) | ||||||
| Method | ELECTRON MICROSCOPY / single particle reconstruction / cryo EM / Resolution: 4.04 Å | ||||||
Authors | Parey, K. / Vonck, J. | ||||||
| Funding support | Germany, 1items
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Citation | Journal: Sci Adv / Year: 2019Title: High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease. Authors: 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 / ![]() 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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Structure visualization
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| Structure viewer | Molecule: Molmil Jmol/JSmol |
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Downloads & links
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Download
| PDBx/mmCIF format | 6rfs.cif.gz | 1.3 MB | Display | PDBx/mmCIF format |
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| PDB format | pdb6rfs.ent.gz | 1.1 MB | Display | PDB format |
| PDBx/mmJSON format | 6rfs.json.gz | Tree view | PDBx/mmJSON format | |
| Others | Other downloads |
-Validation report
| Summary document | 6rfs_validation.pdf.gz | 1.5 MB | Display | wwPDB validaton report |
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| Full document | 6rfs_full_validation.pdf.gz | 1.5 MB | Display | |
| Data in XML | 6rfs_validation.xml.gz | 181.7 KB | Display | |
| Data in CIF | 6rfs_validation.cif.gz | 290.1 KB | Display | |
| Arichive directory | https://data.pdbj.org/pub/pdb/validation_reports/rf/6rfs ftp://data.pdbj.org/pub/pdb/validation_reports/rf/6rfs | HTTPS FTP |
-Related structure data
| Related structure data | ![]() 4874MC ![]() 4872C ![]() 4873C ![]() 6rfqC ![]() 6rfrC M: map data used to model this data C: citing same article ( |
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| Similar structure data |
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Links
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Assembly
| Deposited unit | ![]()
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Components
+Protein , 39 types, 39 molecules ABCDEFGHIJKLMPRSUWXZabcdefghij...
-Acyl carrier protein ... , 2 types, 2 molecules OQ
| #14: Protein | Mass: 12053.585 Da / Num. of mol.: 1 / Source method: isolated from a natural source / Source: (natural) Yarrowia lipolytica (yeast) / References: UniProt: A0A1H6PXT9, UniProt: Q6C926*PLUS |
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| #16: Protein | Mass: 14444.458 Da / Num. of mol.: 1 / Source method: isolated from a natural source / Source: (natural) Yarrowia lipolytica (yeast) / References: UniProt: A0A1D8NG21, UniProt: Q6C7X2*PLUS |
-Non-polymers , 6 types, 12 molecules 










| #42: Chemical | ChemComp-SF4 / #43: Chemical | #44: Chemical | ChemComp-FMN / | #45: Chemical | ChemComp-NDP / | #46: Chemical | ChemComp-ZN / | #47: Chemical | ChemComp-ZMP / | |
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-Details
| Has protein modification | Y |
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-Experimental details
-Experiment
| Experiment | Method: ELECTRON MICROSCOPY |
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| EM experiment | Aggregation state: PARTICLE / 3D reconstruction method: single particle reconstruction |
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Sample preparation
| Component | Name: Mitochondrial NADH:ubiquinone oxidoreductase / Type: COMPLEX / Entity ID: #1-#41 / Source: NATURAL | |||||||||||||||||||||||||
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| Molecular weight | Value: 1 MDa / Experimental value: NO | |||||||||||||||||||||||||
| Source (natural) | Organism: Yarrowia lipolytica (yeast) | |||||||||||||||||||||||||
| Buffer solution | pH: 7.2 | |||||||||||||||||||||||||
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| Specimen | Conc.: 2.7 mg/ml / Embedding applied: NO / Shadowing applied: NO / Staining applied: NO / Vitrification applied: YES | |||||||||||||||||||||||||
| Vitrification | Instrument: FEI VITROBOT MARK IV / Cryogen name: ETHANE / Humidity: 80 % / Chamber temperature: 283 K |
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Electron microscopy imaging
| Experimental equipment | ![]() Model: Tecnai Polara / Image courtesy: FEI Company |
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| Microscopy | Model: FEI POLARA 300 |
| Electron gun | Electron source: FIELD EMISSION GUN / Accelerating voltage: 300 kV / Illumination mode: FLOOD BEAM |
| Electron lens | Mode: BRIGHT FIELD / Nominal magnification: 200000 X / Calibrated magnification: 45872 X / Nominal defocus max: -3000 nm / Nominal defocus min: -2000 nm / Cs: 2 mm / Alignment procedure: COMA FREE |
| Specimen holder | Cryogen: NITROGEN |
| Image recording | Average exposure time: 8 sec. / Electron dose: 60 e/Å2 / Detector mode: COUNTING / Film or detector model: GATAN K2 SUMMIT (4k x 4k) / Num. of grids imaged: 1 / Num. of real images: 5964 |
| EM imaging optics | Energyfilter name: GIF Bioquantum / Energyfilter slit width: 20 eV |
| Image scans | Movie frames/image: 40 |
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Processing
| Software | Name: PHENIX / Version: 1.12_2829: / Classification: refinement | ||||||||||||||||||||||||||||||||||||
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| EM software |
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| CTF correction | Type: PHASE FLIPPING AND AMPLITUDE CORRECTION | ||||||||||||||||||||||||||||||||||||
| Particle selection | Num. of particles selected: 418740 | ||||||||||||||||||||||||||||||||||||
| Symmetry | Point symmetry: C1 (asymmetric) | ||||||||||||||||||||||||||||||||||||
| 3D reconstruction | Resolution: 4.04 Å / Resolution method: FSC 0.143 CUT-OFF / Num. of particles: 145767 / Symmetry type: POINT | ||||||||||||||||||||||||||||||||||||
| Atomic model building | Protocol: OTHER / Space: REAL | ||||||||||||||||||||||||||||||||||||
| Refinement | Highest resolution: 4.04 Å | ||||||||||||||||||||||||||||||||||||
| Refine LS restraints |
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About Yorodumi



Yarrowia lipolytica (yeast)
Germany, 1items
Citation

UCSF Chimera



















PDBj

















