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Open data
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Basic information
| Entry | ![]()  | |||||||||
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| Title | CI protomer-2 membrane arm focus refined map | |||||||||
 Map data | CI (2) membrane arm focus refined map | |||||||||
 Sample | 
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 Keywords | Super complex XL (CI2+CIII2) / ELECTRON TRANSPORT | |||||||||
| Biological species | ![]()  | |||||||||
| Method | single particle reconstruction / cryo EM / Resolution: 3.82 Å | |||||||||
 Authors | Letts JA / Padavannil A | |||||||||
| Funding support |   United States, 1 items 
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 Citation |  Journal: Cell Metab / Year: 2025Title: Formation of I+III supercomplex rescues respiratory chain defects. Authors: Chao Liang / Abhilash Padavannil / Shan Zhang / Sheryl Beh / David R L Robinson / Jana Meisterknecht / Alfredo Cabrera-Orefice / Timothy R Koves / Chika Watanabe / Miyuki Watanabe / María ...Authors: Chao Liang / Abhilash Padavannil / Shan Zhang / Sheryl Beh / David R L Robinson / Jana Meisterknecht / Alfredo Cabrera-Orefice / Timothy R Koves / Chika Watanabe / Miyuki Watanabe / María Illescas / Radiance Lim / Jordan M Johnson / Shuxun Ren / Ya-Jun Wu / Dennis Kappei / Anna Maria Ghelli / Katsuhiko Funai / Hitoshi Osaka / Deborah Muoio / Cristina Ugalde / Ilka Wittig / David A Stroud / James A Letts / Lena Ho /             ![]() Abstract: Mitochondrial electron transport chain (ETC) complexes partition between free complexes and quaternary assemblies known as supercomplexes (SCs). However, the physiological requirement for SCs and the ...Mitochondrial electron transport chain (ETC) complexes partition between free complexes and quaternary assemblies known as supercomplexes (SCs). However, the physiological requirement for SCs and the mechanisms regulating their formation remain controversial. Here, we show that genetic perturbations in mammalian ETC complex III (CIII) biogenesis stimulate the formation of a specialized extra-large SC (SC-XL) with a structure of I+III, resolved at 3.7 Å by cryoelectron microscopy (cryo-EM). SC-XL formation increases mitochondrial cristae density, reduces CIII reactive oxygen species (ROS), and sustains normal respiration despite a 70% reduction in CIII activity, effectively rescuing CIII deficiency. Consequently, inhibiting SC-XL formation in CIII mutants using the Uqcrc1 contact site mutation leads to respiratory decompensation. Lastly, SC-XL formation promotes fatty acid oxidation (FAO) and protects against ischemic heart failure in mice. Our study uncovers an unexpected plasticity in the mammalian ETC, where structural adaptations mitigate intrinsic perturbations, and suggests that manipulating SC-XL formation is a potential therapeutic strategy for mitochondrial dysfunction.  | |||||||||
| History | 
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Structure visualization
| Supplemental images | 
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Downloads & links
-EMDB archive
| Map data |  emd_42127.map.gz | 1.1 GB |  EMDB map data format | |
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| Header (meta data) |  emd-42127-v30.xml emd-42127.xml | 20.7 KB 20.7 KB  | Display Display  |  EMDB header | 
| FSC (resolution estimation) |  emd_42127_fsc.xml | 22.3 KB | Display |  FSC data file | 
| Images |  emd_42127.png | 127.5 KB | ||
| Masks |  emd_42127_msk_1.map | 1.1 GB |  Mask map | |
| Filedesc metadata |  emd-42127.cif.gz | 4.1 KB | ||
| Others |  emd_42127_half_map_1.map.gz emd_42127_half_map_2.map.gz | 1 GB 1 GB  | ||
| Archive directory |  http://ftp.pdbj.org/pub/emdb/structures/EMD-42127 ftp://ftp.pdbj.org/pub/emdb/structures/EMD-42127 | HTTPS FTP  | 
-Validation report
| Summary document |  emd_42127_validation.pdf.gz | 1.2 MB | Display |  EMDB validaton report | 
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| Full document |  emd_42127_full_validation.pdf.gz | 1.2 MB | Display | |
| Data in XML |  emd_42127_validation.xml.gz | 32.2 KB | Display | |
| Data in CIF |  emd_42127_validation.cif.gz | 43.1 KB | Display | |
| Arichive directory |  https://ftp.pdbj.org/pub/emdb/validation_reports/EMD-42127 ftp://ftp.pdbj.org/pub/emdb/validation_reports/EMD-42127 | HTTPS FTP  | 
-Related structure data
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Links
| EMDB pages |  EMDB (EBI/PDBe) /  EMDataResource | 
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Map
| File |  Download / File: emd_42127.map.gz / Format: CCP4 / Size: 1.1 GB / Type: IMAGE STORED AS FLOATING POINT NUMBER (4 BYTES) | ||||||||||||||||||||||||||||||||||||
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| Annotation | CI (2) membrane arm focus refined map | ||||||||||||||||||||||||||||||||||||
| Projections & slices | Image control
 
 Images are generated by Spider.  | ||||||||||||||||||||||||||||||||||||
| Voxel size | X=Y=Z: 0.88 Å | ||||||||||||||||||||||||||||||||||||
| Density | 
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| Symmetry | Space group: 1 | ||||||||||||||||||||||||||||||||||||
| Details | EMDB XML: 
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-Supplemental data
-Mask #1
| File |  emd_42127_msk_1.map | ||||||||||||
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| Projections & Slices | 
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| Density Histograms | 
-Half map: CI (2) membrane arm focus refined half map A
| File | emd_42127_half_map_1.map | ||||||||||||
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| Annotation | CI (2) membrane arm focus refined half map A | ||||||||||||
| Projections & Slices | 
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| Density Histograms | 
-Half map: CI (2) membrane arm focus refined half map A
| File | emd_42127_half_map_2.map | ||||||||||||
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| Annotation | CI (2) membrane arm focus refined half map A | ||||||||||||
| Projections & Slices | 
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| Density Histograms | 
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Sample components
-Entire : Mitochondrial Super-complex with CI dimer and CIII dimer (Super-c...
| Entire | Name: Mitochondrial Super-complex with CI dimer and CIII dimer (Super-complex XL) | 
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| Components | 
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-Supramolecule #1: Mitochondrial Super-complex with CI dimer and CIII dimer (Super-c...
| Supramolecule | Name: Mitochondrial Super-complex with CI dimer and CIII dimer (Super-complex XL) type: complex / ID: 1 / Parent: 0 Macromolecule list: #1, #57, #2-#3, #55, #54, #56, #4, #58, #5-#53  | 
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| Source (natural) | Organism: ![]()  | 
-Experimental details
-Structure determination
| Method | cryo EM | 
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 Processing | single particle reconstruction | 
| Aggregation state | particle | 
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Sample preparation
| Buffer | pH: 7.7 | 
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| Vitrification | Cryogen name: ETHANE | 
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Electron microscopy
| Microscope | TFS GLACIOS | 
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| Image recording | Film or detector model: GATAN K3 (6k x 4k) / Average electron dose: 60.0 e/Å2 | 
| Electron beam | Acceleration voltage: 200 kV / Electron source:  FIELD EMISSION GUN | 
| Electron optics | Illumination mode: OTHER / Imaging mode: BRIGHT FIELD / Nominal defocus max: 3.0 µm / Nominal defocus min: 0.5 µm | 
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About Yorodumi




Keywords
Authors
United States, 1 items 
Citation
 
 
 
 
 







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Processing
FIELD EMISSION GUN

