8GS8

cryo-EM structure of the human respiratory complex II

Summary for 8GS8

• Entry DOI: 10.2210/pdb8gs8/pdb
• EMDB information: 34225
• Descriptor: 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)
• Functional Keywords: succinate dehydrogenase, electron transport chain, human mitochondria, oxidoreductase, oxidative phosphorylation
• Biological source: Homo sapiens (human); More
• Total number of polymer chains: 4
• Total formula weight: 143353.08

Authors

Du, Z.,Zhou, X.,Lai, Y.,Xu, J.,Zhang, Y.,Zhou, S.,Liu, F.,Gao, Y.,Gong, H.,Rao, Z. (deposition date: 2022-09-05, release date: 2023-05-10, Last modification date: 2025-07-02)

Primary citation

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

Experimental method

ELECTRON MICROSCOPY (2.86 Å)