TY  - JOUR
A1  - Parey, Kristian Kurt
A1  - Haapanen, Outi
A1  - Sharma, Vivek
A1  - Köfeler, Harald
A1  - Züllig, Thomas
A1  - Prinz, Simone
A1  - Siegmund, Karin
A1  - Wittig, Ilka
A1  - Mills, Deryck J.
A1  - Vonck, Janet
A1  - Kühlbrandt, Werner
A1  - Zickermann, Volker
T1  - High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease
T2  - Science advances
N2  - 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.
Y1  - 2019
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/54323
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-543232
SN  - 2375-2548
N1  - Distributed under a Creative Commons Attribution NonCommercial License 4.0 (CC BY-NC).
VL  - 5
IS  - 12, eaax9484
SP  - 1
EP  - 10
PB  - Assoc.
CY  - Washington, DC [u. a.]
ER  -