Oxidative Phosphorylation at the fin de siècle, Science, vol.283, issue.5407, pp.1488-1493, 1999. ,
THE ATP SYNTHASE?A SPLENDID MOLECULAR MACHINE, Annual Review of Biochemistry, vol.66, issue.1, pp.717-749, 1997. ,
Structure of ATP synthase from Paracoccus denitrificans determined by X-ray crystallography at 4.0 Å resolution, Proceedings of the National Academy of Sciences, vol.112, issue.43, pp.13231-13236, 2015. ,
Structure and conformational states of the bovine mitochondrial ATP synthase by cryo-EM, eLife, vol.4, 2015. ,
Horizontal membrane-intrinsic ?-helices in the stator a-subunit of an F-type ATP synthase, Nature, vol.521, issue.7551, pp.237-240, 2015. ,
Atomic model for the dimeric FO region of mitochondrial ATP synthase, Science, vol.358, issue.6365, pp.936-940, 2017. ,
Structure of a Complete ATP Synthase Dimer Reveals the Molecular Basis of Inner Mitochondrial Membrane Morphology, Molecular Cell, vol.63, issue.3, pp.445-456, 2016. ,
Purification, characterization and crystallization of the F-ATPase from Paracoccus denitrificans, Open Biology, vol.5, issue.9, p.150119, 2015. ,
High-resolution cryo-EM analysis of the yeast ATP synthase in a lipid membrane, Science, vol.360, issue.6389, p.eaas9699, 2018. ,
ATP Synthase Diseases of Mitochondrial Genetic Origin, Frontiers in Physiology, vol.9, p.329, 2018. ,
URL : https://hal.archives-ouvertes.fr/hal-02398397
Mitochondrial diseases and genetic defects of ATP synthase, Biochim. Biophys. Acta, vol.1757, pp.1400-1405, 2006. ,
Pathogenesis of primary defects in mitochondrial ATP synthesis, Seminars in Cell & Developmental Biology, vol.12, issue.6, pp.441-448, 2001. ,
A Yeast Model of the Neurogenic Ataxia Retinitis Pigmentosa (NARP) T8993G Mutation in the Mitochondrial ATP Synthase-6 Gene, Journal of Biological Chemistry, vol.282, issue.47, pp.34039-34047, 2007. ,
URL : https://hal.archives-ouvertes.fr/hal-00173414
Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients, Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, vol.1793, issue.5, pp.817-824, 2009. ,
URL : https://hal.archives-ouvertes.fr/hal-00366403
Introducing the human Leigh syndrome mutation T9176G into Saccharomyces cerevisiae mitochondrial DNA leads to severe defects in the incorporation of Atp6p into the ATP synthase and in the mitochondrial morphology, Human Molecular Genetics, vol.18, issue.15, pp.2889-2898, 2009. ,
Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1797, issue.6-7, pp.1105-1112, 2010. ,
URL : https://hal.archives-ouvertes.fr/hal-00450957
Defining the pathogenesis of human mtDNA mutations using a yeast model: The case of T8851C, The International Journal of Biochemistry & Cell Biology, vol.45, issue.1, pp.130-140, 2013. ,
URL : https://hal.archives-ouvertes.fr/hal-00735756
Defining the impact on yeast ATP synthase of two pathogenic human mitochondrial DNA mutations, T9185C and T9191C, Biochimie, vol.100, pp.200-206, 2014. ,
URL : https://hal.archives-ouvertes.fr/hal-00940330
Yeast models of mutations in the mitochondrial ATP6 gene found in human cancer cells, Mitochondrion, vol.29, pp.7-17, 2016. ,
, Social Interest/Social Feeling, Encyclopedia of Personality and Individual Differences, vol.2020, pp.5083-5083, 2020.
Identification of G8969>A in mitochondrial ATP6 gene that severely compromises ATP synthase function in a patient with IgA nephropathy, Scientific Reports, vol.6, issue.1, 2016. ,
Genetic transformation of Saccharomyces cerevisiae mitochondria, Methods in Cell Biology, vol.65, pp.381-396, 2001. ,
URL : https://hal.archives-ouvertes.fr/hal-00167435
The Sorting of Mitochondrial DNA and Mitochondrial Proteins in Zygotes: Preferential Transmission of Mitochondrial DNA to the Medial Bud, Journal of Cell Biology, vol.142, issue.3, pp.613-623, 1998. ,
Two New Mutations in theMTATP6Gene Associated with Leigh Syndrome, Neuropediatrics, vol.36, issue.5, pp.314-318, 2005. ,
NH2-terminal sequence of the isolated yeast ATP synthase subunit 6 reveals post-translational cleavage, European Journal of Biochemistry, vol.172, issue.3, pp.621-625, 1988. ,
URL : https://hal.archives-ouvertes.fr/hal-02692275
Isolation and RNA sequence analysis of cytochrome b mutants resistant to funiculosin, a center i inhibitor of the mitochondrial ubiquinol-cytochrome c reductase in Saccharomyces cerevisiae, FEBS Letters, vol.263, issue.1, pp.93-98, 1990. ,
Level of ATP synthase activity required for yeast Saccharomyces cerevisiae to grow on glycerol media, FEBS Letters, vol.343, issue.2, pp.160-164, 1994. ,
Experimental Relocation of the Mitochondrial ATP9 Gene to the Nucleus Reveals Forces Underlying Mitochondrial Genome Evolution, PLoS Genetics, vol.8, issue.8, p.e1002876, 2012. ,
URL : https://hal.archives-ouvertes.fr/hal-00735337
Maintenance and Integrity of the Mitochondrial Genome: a Plethora of Nuclear Genes in the Budding Yeast, Microbiology and Molecular Biology Reviews, vol.64, issue.2, pp.281-315, 2000. ,
Yeast Cells Lacking the Mitochondrial Gene Encoding the ATP Synthase Subunit 6 Exhibit a Selective Loss of Complex IV and Unusual Mitochondrial Morphology, Journal of Biological Chemistry, vol.282, issue.15, pp.10853-10864, 2007. ,
Deregulating mitochondrial metabolite and ion transport has beneficial effects in yeast and human cellular models for NARP syndrome, Human Molecular Genetics, vol.28, issue.22, pp.3792-3804, 2019. ,
URL : https://hal.archives-ouvertes.fr/hal-02362008
Investigation of the Role and Mechanism of IF1 and STF1 Proteins, Twin Inhibitory Peptides Which Interact with the Yeast Mitochondrial ATP Synthase, Biochemistry, vol.42, issue.24, pp.7626-7636, 2003. ,
Cryo-EM of ATP synthases, Current Opinion in Structural Biology, vol.52, pp.71-79, 2018. ,
Modular assembly of yeast mitochondrial ATP synthase, The EMBO Journal, vol.30, issue.5, pp.920-930, 2011. ,
Atp10p Assists Assembly of Atp6p into the F0Unit of the Yeast Mitochondrial ATPase, Journal of Biological Chemistry, vol.279, issue.19, pp.19775-19780, 2004. ,
A Single Amino Acid Change in Subunit 6 of the Yeast Mitochondrial ATPase Suppresses a Null Mutation inATP10, Journal of Biological Chemistry, vol.275, issue.38, pp.29238-29243, 2000. ,
Identification of a Nuclear Gene (FMC1) Required for the Assembly/Stability of Yeast Mitochondrial F1-ATPase in Heat Stress Conditions, Journal of Biological Chemistry, vol.276, issue.9, pp.6789-6796, 2000. ,
OGlcNAcylation and Phosphorylation Have Similar Structural Effects in ?-Helices: Post-Translational Modifications as Inducible Start and Stop Signals in ?-Helices, with Greater Structural Effects on Threonine Modification, Biochemistry, vol.53, issue.14, pp.2242-2260, 2014. ,
The complete sequence of the mitochondrial genome of Saccharomyces cerevisiae, FEBS Letters, vol.440, issue.3, pp.325-331, 1998. ,
Codon recognition rules in yeast mitochondria., Proceedings of the National Academy of Sciences, vol.77, issue.6, pp.3167-3170, 1980. ,
The pathogenic MT-ATP6 m.8851T>C mutation prevents proton movements within the n-side hydrophilic cleft of the membrane domain of ATP synthase, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1860, issue.7, pp.562-572, 2019. ,
URL : https://hal.archives-ouvertes.fr/hal-02404946
Molecular basis of diseases caused by the mtDNA mutation m.8969G>A in the subunit a of ATP synthase, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.1859, issue.8, pp.602-611, 2018. ,
[19] Preparation of yeast mitochondria (Saccharomyces cerevisiae) with good P/O and respiratory control ratios, Biomembranes Part F: Bioenergetics: Oxidative Phosphorylation, vol.55, pp.149-159, 1979. ,
Protein measurement with the Folin phenol reagent, J. Biol. Chem, vol.193, pp.265-275, 1951. ,
Phosphate transport and ATP synthesis in yeast mitochondria, FEBS Letters, vol.102, issue.1, pp.18-22, 1979. ,
Rhodamine 123 as a probe of transmembrane potential in isolated rat-liver mitochondria: spectral and metabolic properties, Biochimica et Biophysica Acta (BBA) - Bioenergetics, vol.850, issue.3, pp.436-448, 1986. ,
Induction and Repression of Mitochondrial ATPase in Yeast, European Journal of Biochemistry, vol.5, issue.2, pp.276-284, 1968. ,
An improved assay for nanomole amounts of inorganic phosphate, Analytical Biochemistry, vol.100, issue.1, pp.95-97, 1979. ,
Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form, Analytical Biochemistry, vol.199, issue.2, pp.223-231, 1991. ,
Separation by Blue Native and Colorless Native Polyacrylamide Gel Electrophoresis of the Oxidative Phosphorylation Complexes of Yeast Mitochondria Solubilized by Different Detergents: Specific Staining of the Different Complexes, Analytical Biochemistry, vol.242, issue.2, pp.248-254, 1996. ,
ATP Synthase of Yeast Mitochondria, Journal of Biological Chemistry, vol.271, issue.34, pp.20284-20290, 1996. ,
Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4, Nature, vol.227, issue.5259, pp.680-685, 1970. ,
Quantifying Western blots: Pitfalls of densitometry, ELECTROPHORESIS, vol.30, issue.11, pp.1845-1855, 2009. ,
Fast, scalable generation of high?quality protein multiple sequence alignments using Clustal Omega, Molecular Systems Biology, vol.7, issue.1, p.539, 2011. ,
The PyMOL Molecular Graphics System, Version 0.99, Schrödinger, LLC, p.17, 2002. ,
, Figure 2?figure supplement 1. Representative images used in the experiment, sampled from http://commons.wikimedia.org/wiki/Main_Page under the Creative Commons Attribution 4.0 International Public License https://creativecommons.org/licenses/by/4.0/., © 2020 by the authors. Licensee MDPI