The energetic state of mitochondria modulates complex III biogenesis through the atp-dependent activity of bcs1
Cell Metabolism. 2013-10-01; 18(4): 567-577
DOI: 10.1016/j.cmet.2013.08.017
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1. Cell Metab. 2013 Oct 1;18(4):567-77. doi: 10.1016/j.cmet.2013.08.017. Epub 2013
Sep 19.
The energetic state of mitochondria modulates complex III biogenesis through the
ATP-dependent activity of Bcs1.
Ostojić J(1), Panozzo C, Lasserre JP, Nouet C, Courtin F, Blancard C, di Rago JP,
Dujardin G.
Author information:
(1)Centre de Génétique Moléculaire, Université Paris-Sud, avenue de la Terrasse,
91198 Gif sur Yvette, France.
Comment in
Microb Cell. 2014 Jan 06;1(1):43-44.
Our understanding of the mechanisms involved in mitochondrial biogenesis has
continuously expanded during the last decades, yet little is known about how they
are modulated to optimize the functioning of mitochondria. Here, we show that
mutations in the ATP binding domain of Bcs1, a chaperone involved in the assembly
of complex III, can be rescued by mutations that decrease the ATP hydrolytic
activity of the ATP synthase. Our results reveal a Bcs1-mediated control loop in
which the biogenesis of complex III is modulated by the energy-transducing
activity of mitochondria. Although ATP is well known as a regulator of a number
of cellular activities, we show here that ATP can be also used to modulate the
biogenesis of an enzyme by controlling a specific chaperone involved in its
assembly. Our study further highlights the intramitochondrial adenine nucleotide
pool as a potential target for the treatment of Bcs1-based disorders.
Copyright © 2013 Elsevier Inc. All rights reserved.
DOI: 10.1016/j.cmet.2013.08.017
PMID: 24055101 [Indexed for MEDLINE]