A novel mechanism involved in the coupling of mitochondrial biogenesis to oxidative phosphorylation

Abstract

Mitochondria are essential organelles that are central to a multitude of cellular processes, including oxidative phosphorylation (OXPHOS), which produces most of the ATP in animal cells. Thus it is important to understand not only the mechanisms and biogenesis of this energy production machinery but also how it is regulated in both physiological and pathological contexts. A recent study by Ostojić et al. [Cell Metabolism (2013) 18, 567-577] has uncovered a regulatory loop by which the biogenesis of a major enzyme of the OXPHOS pathway, the respiratory complex III, is coupled to the energy producing activity of the mitochondria.

Keywords

• yeast
• mitochondrial-disease model
• AAA protein Bcs1
• OXPHOS complex III
• ATP synthase