Mitochondrial cytochrome c oxidase biogenesis: Recent developments.

Timón-Gómez A; Nývltová E; Abriata LA; Vila AJ; Hosler J; Barrientos A

doi:10.1016/j.semcdb.2017.08.055

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Journal article

Mitochondrial cytochrome c oxidase biogenesis: Recent developments.

Timón-Gómez A Department of Neurology, University of Miami Miller School of Medicine, Miami, FL, United States.
Nývltová E Department of Neurology, University of Miami Miller School of Medicine, Miami, FL, United States.
Abriata LA Laboratory for Biomolecular Modeling & Protein Purification and Structure Facility, École Polytechnique Fédérale de Lausanne and Swiss Institute of Bioinformatics, Switzerland.
Vila AJ Instituto de Biología Molecular y Celular de Rosario (IBR, CONICET-UNR), Ocampo y Esmeralda, S2002LRK Rosario, Argentina.
Hosler J Department of Biochemistry, The University of Mississippi Medical Center, Jackson, MS, United States.
Barrientos A Department of Neurology, University of Miami Miller School of Medicine, Miami, FL, United States; Department of Biochemistry and Molecular Biology, University of Miami Miller School of Medicine, Miami, FL, United States. Electronic address: abarrientos@med.miami.edu.

2017-09-06

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Seminars in cell & developmental biology. - 2018

Mitochondrial cytochrome c oxidase

English Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen consumption and is essential for aerobic energy generation in the form of ATP. Human COX is a copper-heme A hetero-multimeric complex formed by 3 catalytic core subunits encoded in the mitochondrial DNA and 11 subunits encoded in the nuclear genome. Investigations over the last 50 years have progressively shed light into the sophistication surrounding COX biogenesis and the regulation of this process, disclosing multiple assembly factors, several redox-regulated processes leading to metal co-factor insertion, regulatory mechanisms to couple synthesis of COX subunits to COX assembly, and the incorporation of COX into respiratory supercomplexes. Here, we will critically summarize recent progress and controversies in several key aspects of COX biogenesis: linear versus modular assembly, the coupling of mitochondrial translation to COX assembly and COX assembly into respiratory supercomplexes.

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