Parey 2019 Sci Adv

From Bioblast
Publications in the MiPMap
Parey K, Haapanen O, Sharma V, KΓΆfeler H, ZΓΌllig T, Prinz S, Siegmund K, Wittig I, Mills DJ, Vonck J, KΓΌhlbrandt W, Zickermann V (2019) High-resolution cryo-EM structures of respiratory complex I: Mechanism, assembly, and disease.

Β» Sci Adv 5:eaax9484. PMID: 31844670 Open Access

Parey Kristian, Haapanen Outi, Sharma Vivek, Koefeler Harald, Zuellig Thomas, Prinz Simone, Siegmund Karin, Wittig Ilka, Mills Deryck J, Vonck Janet, Kuehlbrandt Werner, Zickermann Volker (2019) Sci Adv

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 Yarrowia lipolytica by electron cryo-microscopy at 3.2-Γ… 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.

β€’ Bioblast editor: Plangger M

Labels: MiParea: mt-Structure;fission;fusion 

Enzyme: Complex I 

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