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The energetic state of mitochondria modulates complex III biogenesis through the ATP-dependent activity of Bcs1.

Authors
  • Ostojić, Jelena1
  • Panozzo, Cristina
  • Lasserre, Jean-Paul
  • Nouet, Cécile
  • Courtin, Florence
  • Blancard, Corinne
  • di Rago, Jean-Paul
  • Dujardin, Geneviève
  • 1 Centre de Génétique Moléculaire, Université Paris-Sud, avenue de la Terrasse, 91198 Gif sur Yvette, France. , (France)
Type
Published Article
Journal
Cell metabolism
Publication Date
Oct 01, 2013
Volume
18
Issue
4
Pages
567–577
Identifiers
DOI: 10.1016/j.cmet.2013.08.017
PMID: 24055101
Source
Medline
License
Unknown

Abstract

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.

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