Journal
SCIENCE ADVANCES
Volume 5, Issue 12, Pages -Publisher
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/sciadv.aax9484
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Funding
- Deutsche Forschungsgemeinschaft [ZI 552/4-2, SFB 815/Z1]
- Academy of Finland [294652]
- University of Helsinki
- Sigrid Juselius Foundation
- CHEMS doctoral school of the University of Helsinki
- Austrian Federal Ministry of Education, Science and Research [BMWFW-10.420/0005-WF/V/3c/2017]
- BMBF mitoNET-German Network for Mitochondrial Disorders [01GM1906D]
- Max Planck Society
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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-angstrom 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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