Supercomplex Assembly Determines Electron Flux in the Mitochondrial Electron Transport Chain

By Esther Lapuente-Brun, R. Moreno-Loshuertos, R. Acín-Pérez, Ana Latorre-Pellicer, C. Colás, Eduardo Balsa, Ester Perales-Clemente, Pedro M. Quirós, E. Calvo, M. A. Rodríguez-Hernández and 8 more. Science, 2013

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Respiration Refined Cells derive energy from redox reactions mediated by mitochondrial enzymes that form the electron transport chain. The enzymes can form large complexes, known as supercomplexes, whose function has been controversial. Lapuente-Brun et al. (p. 1567) discovered that a mouse protein, supercomplex assembly factor I (SCAFI), specifically modulates assembly of respiratory complexes into supercomplexes. Formation of the supercomplexes appears to cause electrons to be processed differently, depending on the substrate from which they are derived. Ordered formation of supercomplexes of respiratory enzymes influences metabolic efficiency in response to food supply. The textbook description of mitochondrial respiratory complexes (RCs) views them as free-moving entities linked by the mobile carriers coenzyme Q (CoQ) and cytochrome c (cyt c). This model (known as the fluid model) is challenged by the proposal that all RCs except complex II can associate in supercomplexes (SCs). The proposed SCs are the respirasome (complexes I, III, and IV), complexes I and III, and complexes III and IV. The role of SCs is unclear, and their existence is debated. By genetic modulation of interactions between complexes I and III and III and IV, we show that these associations define dedicated CoQ and cyt c pools and that SC assembly is dynamic and organizes electron flux to optimize the use of available substrates.

Subfields: Clinical Biochemistry, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/4b6acd787352670a5af105382716d34596f26e90

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Citations in the corpus

Mitochondrial Cristae Shape Determines Respiratory Chain Supercomplexes Assembly and Respiratory Efficiency (2013, background): “1Dulbecco-Telethon Institute, Venetian Institute of Molecular Medicine, Via Orus 2, 35129 Padova, Italy 2Department of Biology, University of Padova, Via U. Bassi 58B, 35121 Padova, Italy 3IRCCS Fondazione Santa Lucia, Via Ardeatina 306, 00143 Rome, Italy 4Clinical Genetics Unit, Department of Woman”
Mitochondrial function as a therapeutic target in heart failure (2016, neutral): “…been observed in animal models 129 and humans 35 with HF. Electron transport system proteins seem to aggregate into functional supercomplexes 130–132 , and a loss of mitochondrial supercomplexes, which is postulated to have a causal role in mitochondrial ROS generation 133 , has been noted in…”