7ZKP

Late assembly intermediate of the proximal proton pumping module of complex I with assembly factors NDUFAF1 and CIA84

Summary for 7ZKP

• Entry DOI: 10.2210/pdb7zkp/pdb
• EMDB information: 14764
• Descriptor: Subunit NIMM of NADH:Ubiquinone Oxidoreductase (Complex I), subunit NI9M of protein NADH:Ubiquinone Oxidoreductase (Complex I), Subunit NEBM of NADH:Ubiquinone Oxidoreductase (Complex I), ... (19 entities in total)
• Functional Keywords: assembly respiratory chain membrane protein mitochondria, electron transport
• Biological source: Yarrowia lipolytica; More
• Total number of polymer chains: 14
• Total formula weight: 361535.91

Authors

Schiller, J.,Laube, E.,Vonck, J.,Zickermann, V. (deposition date: 2022-04-13, release date: 2022-11-23, Last modification date: 2024-11-13)

Primary citation

Schiller, J.,Laube, E.,Wittig, I.,Kuhlbrandt, W.,Vonck, J.,Zickermann, V.
Insights into complex I assembly: Function of NDUFAF1 and a link with cardiolipin remodeling.
Sci Adv, 8:eadd3855-eadd3855, 2022

PubMed Abstract: Respiratory complex I is a ~1-MDa proton pump in mitochondria. Its structure has been revealed in great detail, but the structural basis of its assembly, in humans involving at least 15 assembly factors, is essentially unknown. We determined cryo-electron microscopy structures of assembly intermediates associated with assembly factor NDUFAF1 in a yeast model system. Subunits ND2 and NDUFC2 together with assembly factors NDUFAF1 and CIA84 form the nucleation point of the NDUFAF1-dependent assembly pathway. Unexpectedly, the cardiolipin remodeling enzyme tafazzin is an integral component of this core complex. In a later intermediate, all 12 subunits of the proximal proton pump module have assembled. NDUFAF1 locks the central ND3 subunit in an assembly-competent conformation, and major rearrangements of central subunits are required for complex I maturation.

PubMed: 36383672
DOI: 10.1126/sciadv.add3855

Experimental method

ELECTRON MICROSCOPY (3.2 Å)