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Regulation of mitochondrial respiration and ATP synthesis via cytochrome c oxidase

  • Current topics in Biology
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Rendiconti Lincei. Scienze Fisiche e Naturali Aims and scope Submit manuscript

Abstract

Cytochrome c oxidase (COX) represents the main oxygen acceptor for respiration of aerobic organisms. The energy of respiration is stored via oxidative phosphorylation (OxPhos) in ATP, the general energy intermediate in living cells, or released as heat. During evolution from bacteria to mammals the complexity of OxPhos regulation increased at COX by increasing the number of subunits, by expression of subunit isoforms which are specific for tissues, developmental stages, and oxygen concentrations, and by reversible phosphorylation. The essential function of the “supernumerary” subunits became evident in patients with mitochondrial disease related to COX-deficiency, based on mutations in these subunit genes. While the basic regulation of energy transduction in OxPhos was explained by the Mitchell theory, an additional regulation of respiration and ATP synthesis was discovered in eukaryotic organisms, based on reversible and membrane potential-independent inhibition of COX activity at high ATP/ADP ratios. So far, only some of the complex regulatory functions of nuclear-encoded subunits were uncovered.

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Fig. 1

Taken from Kadenbach et al. (2000)

Fig. 2

Taken from Ramzan et al. (2010)

Fig. 3

Taken from Helling et al. (2012)

Fig. 4

Taken from Kadenbach et al. (2010)

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Acknowledgements

Funding was provided by Deutsche Forschungsgemeinschaft.

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  1. Fachbereich Chemie, Philipps-Universität, 35032, Marburg, Germany

    Bernhard Kadenbach

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Correspondence to Bernhard Kadenbach.

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This paper belongs to a series of peer-reviewed contributions coordinated by Guest Editor Ferdinando Palmieri on the theme “Current topics in biology”.

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Kadenbach, B. Regulation of mitochondrial respiration and ATP synthesis via cytochrome c oxidase. Rend. Fis. Acc. Lincei 29, 421–435 (2018). https://doi.org/10.1007/s12210-018-0710-y

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