6RFR

Cryo-EM structure of respiratory complex I from Yarrowia lipolytica at 3.2 A resolution

Summary for 6RFR

• Entry DOI: 10.2210/pdb6rfr/pdb
• EMDB information: 4873
• Descriptor: Subunit NUAM of NADH:Ubiquinone Oxidoreductase (Complex I), Subunit NUJM of NADH:Ubiquinone Oxidoreductase (Complex I), Subunit NUKM of NADH:Ubiquinone Oxidoreductase (Complex I), ... (55 entities in total)
• Functional Keywords: complex i, nadh dehydrogenase, mitochondrion proton pumping, ubiquinone, oxidoreductase
• Biological source: Yarrowia lipolytica (Candida lipolytica); More
• Total number of polymer chains: 42
• Total formula weight: 1014891.09

Authors

Parey, K.,Vonck, J. (deposition date: 2019-04-16, release date: 2019-12-11, Last modification date: 2024-10-23)

Primary citation

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

Experimental method

ELECTRON MICROSCOPY (3.2 Å)