High-resolution cryo-EM structures of respiratory complex I : Mechanism, assembly, and disease

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Parey , K , Haapanen , O , Sharma , V , Köfeler , H , Züllig , T , Prinz , S , Siegmund , K , Wittig , I , Mills , D J , Vonck , J , Kühlbrandt , W & Zickermann , V 2019 , ' High-resolution cryo-EM structures of respiratory complex I : Mechanism, assembly, and disease ' , Science Advances , vol. 5 , no. 12 , 9484 . https://doi.org/10.1126/sciadv.aax9484

Title: High-resolution cryo-EM structures of respiratory complex I : Mechanism, assembly, and disease
Author: Parey, Kristian; Haapanen, Outi; Sharma, Vivek; Köfeler, Harald; Züllig, Thomas; Prinz, Simone; Siegmund, Karin; Wittig, Ilka; Mills, Deryck J.; Vonck, Janet; Kühlbrandt, Werner; Zickermann, Volker
Contributor organization: Materials Physics
Department of Physics
Institute of Biotechnology
Date: 2019-12
Language: eng
Number of pages: 10
Belongs to series: Science Advances
ISSN: 2375-2548
DOI: https://doi.org/10.1126/sciadv.aax9484
URI: http://hdl.handle.net/10138/317884
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 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.
Subject: NADH-UBIQUINONE OXIDOREDUCTASE
ACTIVE/DE-ACTIVE TRANSITION
MOLECULAR-DYNAMICS
CRYSTAL-STRUCTURE
FORCE-FIELD
MITOCHONDRIAL
NDUFS4
IDENTIFICATION
SUBUNIT
ORIENTATION
114 Physical sciences
Peer reviewed: Yes
Rights: cc_by_nc
Usage restriction: openAccess
Self-archived version: publishedVersion
Funder: Valtion perusrahoitus/hankkeet
Grant number:


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