Bcs1, a AAA protein of the mitochondria with a role in the biogenesis of the respiratory chain

Journal of Structural Biology
Nikola Wagener, Walter Neupert

Abstract

The family of AAA+ proteins in eukaryotes has many members in various cellular compartments with a broad spectrum of functions in protein unfolding and degradation. The mitochondrial AAA protein Bcs1 plays an unusual role in protein translocation. It is involved in the topogenesis of the Rieske protein, Rip1, and thereby in the biogenesis of the cytochrome bc(1) complex of the mitochondrial respiratory chain. Bcs1 mediates the export of the folded FeS domain of Rip1 across the mitochondrial inner membrane and the insertion of its transmembrane segment into an assembly intermediate of the cytochrome bc(1) complex. We discuss structural elements of the Bcs1 protein compared to other AAA proteins in an attempt to understand the mechanism of its function. In this context, we discuss human diseases caused by mutations in Bcs1 that lead to different properties of the protein and subsequently to different symptoms.

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Citations

Jul 19, 2013·Integrative and Comparative Biology·Walker Pett, Dennis V Lavrov
Aug 26, 2014·Proceedings of the National Academy of Sciences of the United States of America·Alyssa B RosenbloomCarlos Bustamante
Feb 24, 2016·Biochimica Et Biophysica Acta·Lucia BiasuttoMario Zoratti
Feb 17, 2015·Biochimica Et Biophysica Acta·Annalea ConteVincenzo Zara
May 7, 2016·Journal of Hepatology·Laura S KremerAnne Lombès
Mar 3, 2018·International Journal of Molecular Sciences·Renuka KolliChris Carrie
Dec 26, 2018·American Journal of Medical Genetics. Part a·Rachael A BakerPaul R Mark
Feb 20, 2020·Nature Structural & Molecular Biology·Peter S Shen
Jan 29, 2020·Nature Structural & Molecular Biology·Lukas KaterRoland Beckmann
Feb 12, 2020·Nature Structural & Molecular Biology·Wai Kwan TangDi Xia
Mar 3, 2020·Biochimica Et Biophysica Acta. Bioenergetics·Katharina Stephan, Martin Ott

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