6RFQ

Cryo-EM structure of a respiratory complex I assembly intermediate with NDUFAF2

6RFQ の概要

• エントリーDOI: 10.2210/pdb6rfq/pdb
• EMDBエントリー: 4872
• 分子名称: Subunit NUAM of NADH:Ubiquinone Oxidoreductase (Complex I), Subunit NUJM of NADH:Ubiquinone Oxidoreductase (Complex I), Subunit NUKM of NADH:Ubiquinone Oxidoreductase (Complex I), ... (52 entities in total)
• 機能のキーワード: complex i, nadh dehydrogenase, mitochondrion proton pumping, ubiquinone, oxidoreductase
• 由来する生物種: Yarrowia lipolytica (Candida lipolytica); 詳細
• タンパク質・核酸の鎖数: 41
• 化学式量合計: 1000929.80

構造登録者

Parey, K.,Vonck, J. (登録日: 2019-04-16, 公開日: 2018-10-10, 最終更新日: 2025-07-02)

主引用文献

Parey, K.,Haapanen, O.,Sharma, V.,Kofeler, H.,Zullig, T.,Prinz, S.,Siegmund, K.,Wittig, I.,Mills, D.J.,Vonck, J.,Kuhlbrandt, W.,Zickermann, V.
High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease.
Sci Adv, 5:eaax9484-eaax9484, 2019

PubMed 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 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.

PubMed: 31844670
DOI: 10.1126/sciadv.aax9484

実験手法

ELECTRON MICROSCOPY (3.3 Å)