Kristian Kurt Parey, Outi Haapanen, Vivek Sharma, Harald Köfeler, Thomas Züllig, Simone Prinz, Karin Siegmund, Ilka Wittig, Deryck J. Mills, Janet Vonck, Werner Kühlbrandt, Volker Zickermann
- 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-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.
MetadatenAuthor: | Kristian Kurt PareyORCiDGND, Outi HaapanenORCiD, Vivek SharmaORCiD, Harald Köfeler, Thomas Züllig, Simone Prinz, Karin Siegmund, Ilka WittigORCiD, Deryck J. MillsORCiD, Janet VonckORCiD, Werner KühlbrandtORCiDGND, Volker ZickermannORCiDGND |
---|
URN: | urn:nbn:de:hebis:30:3-543232 |
---|
DOI: | https://doi.org/10.1126/sciadv.aax9484 |
---|
ISSN: | 2375-2548 |
---|
Pubmed Id: | https://pubmed.ncbi.nlm.nih.gov/31844670 |
---|
Parent Title (English): | Science advances |
---|
Publisher: | Assoc. |
---|
Place of publication: | Washington, DC [u. a.] |
---|
Document Type: | Article |
---|
Language: | English |
---|
Year of Completion: | 2019 |
---|
Date of first Publication: | 2019/12/11 |
---|
Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
---|
Release Date: | 2020/06/17 |
---|
Volume: | 5 |
---|
Issue: | 12, eaax9484 |
---|
Page Number: | 11 |
---|
First Page: | 1 |
---|
Last Page: | 10 |
---|
Note: | Distributed under a Creative Commons Attribution NonCommercial License 4.0 (CC BY-NC). |
---|
HeBIS-PPN: | 467349940 |
---|
Institutes: | Biochemie, Chemie und Pharmazie / Biochemie und Chemie |
---|
| Biowissenschaften / Biowissenschaften |
---|
| Medizin / Medizin |
---|
| Wissenschaftliche Zentren und koordinierte Programme / Sonderforschungsbereiche / Forschungskollegs |
---|
| Angeschlossene und kooperierende Institutionen / MPI für Biophysik |
---|
Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
---|
Sammlungen: | Universitätspublikationen |
---|
Licence (English): | Creative Commons - Namensnennung-Nicht kommerziell 4.0 |
---|