8GS8

cryo-EM structure of the human respiratory complex II

8GS8 の概要

• エントリーDOI: 10.2210/pdb8gs8/pdb
• EMDBエントリー: 34225
• 分子名称: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial, PROTOPORPHYRIN IX CONTAINING FE, (1S)-2-{[(2-AMINOETHOXY)(HYDROXY)PHOSPHORYL]OXY}-1-[(PALMITOYLOXY)METHYL]ETHYL STEARATE, ... (11 entities in total)
• 機能のキーワード: succinate dehydrogenase, electron transport chain, human mitochondria, oxidoreductase, oxidative phosphorylation
• 由来する生物種: Homo sapiens (human); 詳細
• タンパク質・核酸の鎖数: 4
• 化学式量合計: 143353.08

構造登録者

Du, Z.,Zhou, X.,Lai, Y.,Xu, J.,Zhang, Y.,Zhou, S.,Liu, F.,Gao, Y.,Gong, H.,Rao, Z. (登録日: 2022-09-05, 公開日: 2023-05-10, 最終更新日: 2025-07-02)

主引用文献

Du, Z.,Zhou, X.,Lai, Y.,Xu, J.,Zhang, Y.,Zhou, S.,Feng, Z.,Yu, L.,Tang, Y.,Wang, W.,Yu, L.,Tian, C.,Ran, T.,Chen, H.,Guddat, L.W.,Liu, F.,Gao, Y.,Rao, Z.,Gong, H.
Structure of the human respiratory complex II.
Proc.Natl.Acad.Sci.USA, 120:e2216713120-e2216713120, 2023

PubMed Abstract: Human complex II is a key protein complex that links two essential energy-producing processes: the tricarboxylic acid cycle and oxidative phosphorylation. Deficiencies due to mutagenesis have been shown to cause mitochondrial disease and some types of cancers. However, the structure of this complex is yet to be resolved, hindering a comprehensive understanding of the functional aspects of this molecular machine. Here, we have determined the structure of human complex II in the presence of ubiquinone at 2.86 Å resolution by cryoelectron microscopy, showing it comprises two water-soluble subunits, SDHA and SDHB, and two membrane-spanning subunits, SDHC and SDHD. This structure allows us to propose a route for electron transfer. In addition, clinically relevant mutations are mapped onto the structure. This mapping provides a molecular understanding to explain why these variants have the potential to produce disease.

PubMed: 37098072
DOI: 10.1073/pnas.2216713120

実験手法

ELECTRON MICROSCOPY (2.86 Å)