Gene Symbol: ATP6
Description: F1F0 ATP synthase subunit a
Alias: OLI2, OLI4, PHO1, F1F0 ATP synthase subunit a
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Rak M, Tetaud E, Godard F, Sagot I, Salin B, Duvezin Caubet S, et al. Yeast cells lacking the mitochondrial gene encoding the ATP synthase subunit 6 exhibit a selective loss of complex IV and unusual mitochondrial morphology. J Biol Chem. 2007;282:10853-64 pubmed
    ..accumulation of rho-/rho0 petites issued from large deletions in mtDNA could be restricted to 20-30% by growing the atp6 mutant in media lacking arginine...
  2. Rampelt H, Bohnert M, Zerbes R, Horvath S, Warscheid B, Pfanner N, et al. Mic10, a Core Subunit of the Mitochondrial Contact Site and Cristae Organizing System, Interacts with the Dimeric F1Fo-ATP Synthase. J Mol Biol. 2017;429:1162-1170 pubmed publisher
    ..In addition to its central function in sculpting crista junctions, a fraction of Mic10 molecules interact with the cristae rim-forming F1Fo-ATP synthase. ..
  3. Ostojic J, Panozzo C, Lasserre J, Nouet C, Courtin F, Blancard C, et al. The energetic state of mitochondria modulates complex III biogenesis through the ATP-dependent activity of Bcs1. Cell Metab. 2013;18:567-77 pubmed publisher
    ..Our study further highlights the intramitochondrial adenine nucleotide pool as a potential target for the treatment of Bcs1-based disorders. ..
  4. Fontanesi F, SOTO I, Horn D, Barrientos A. Mss51 and Ssc1 facilitate translational regulation of cytochrome c oxidase biogenesis. Mol Cell Biol. 2010;30:245-59 pubmed publisher
    ..We conclude that Ssc1, by interacting with Mss51 and Mss51-containing complexes, plays a critical role in Cox1 biogenesis, COX assembly, and the translational regulation of these processes. ..
  5. Jia L, Dienhart M, Stuart R. Oxa1 directly interacts with Atp9 and mediates its assembly into the mitochondrial F1Fo-ATP synthase complex. Mol Biol Cell. 2007;18:1897-908 pubmed
    ..observed to assemble into an oligomeric complex containing F(1)-subunits, but its further assembly with subunit 6 (Atp6) of the F(o)-sector was perturbed...
  6. John U, Nagley P. Sequence of the mitochondrial oli2 gene coding for subunit 6 of the mitochondrial ATPase complex in different strains of Saccharomyces. Nucleic Acids Res. 1987;15:366 pubmed
  7. Davies K, Anselmi C, Wittig I, Faraldo Gómez J, Kuhlbrandt W. Structure of the yeast F1Fo-ATP synthase dimer and its role in shaping the mitochondrial cristae. Proc Natl Acad Sci U S A. 2012;109:13602-7 pubmed publisher
  8. Velours J, Stines Chaumeil C, Habersetzer J, Chaignepain S, Dautant A, Brèthes D. Evidence of the proximity of ATP synthase subunits 6 (a) in the inner mitochondrial membrane and in the supramolecular forms of Saccharomyces cerevisiae ATP synthase. J Biol Chem. 2011;286:35477-84 pubmed publisher
  9. Pagadala V, Vistain L, Symersky J, Mueller D. Characterization of the mitochondrial ATP synthase from yeast Saccharomyces cerevisae. J Bioenerg Biomembr. 2011;43:333-47 pubmed publisher
    ..F(1)F(o) ATP synthase with ?-GFP was purified to homogeneity and serves as an excellent enzyme for two- and three-dimensional crystallization studies. ..

More Information


  1. Shingu Vazquez M, Camacho Villasana Y, Sandoval Romero L, Butler C, Fox T, Perez Martinez X. The carboxyl-terminal end of Cox1 is required for feedback assembly regulation of Cox1 synthesis in Saccharomyces cerevisiae mitochondria. J Biol Chem. 2010;285:34382-9 pubmed publisher
    ..Our data suggest a novel activity of Pet54 required for normal synthesis of Cox1 that is independent of the Cox1 C-terminal end. ..
  2. Lau W, Baker L, Rubinstein J. Cryo-EM structure of the yeast ATP synthase. J Mol Biol. 2008;382:1256-64 pubmed publisher
    ..This new map has allowed construction of a model of subunit arrangement in the F(O) motor of ATP synthase that dictates how dimerization of the complex via subunits e and g might occur. ..
  3. Macino G, Tzagoloff A. Assembly of the mitochondrial membrane system: sequence analysis of a yeast mitochondrial ATPase gene containing the oli-2 and oli-4 loci. Cell. 1980;20:507-17 pubmed
    ..Based on its molecular weight, the gene product is tentatively proposed to be either subunit 3 or 6 of the oligomycin-sensitive ATPase. ..
  4. Rak M, Gokova S, Tzagoloff A. Modular assembly of yeast mitochondrial ATP synthase. EMBO J. 2011;30:920-30 pubmed publisher
    ..These studies show that assembly of the ATP synthase is not a single linear process, as previously thought, but rather involves two separate but coordinately regulated pathways that converge at the end stage. ..
  5. Kucharczyk R, Ezkurdia N, Couplan E, Procaccio V, Ackerman S, Blondel M, et al. Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase. Biochim Biophys Acta. 2010;1797:1105-12 pubmed publisher
    ..In vitro, yeast atp6-T9176C mitochondria showed a 30% decrease in the rate of ATP synthesis...
  6. Zeng X, Kucharczyk R, di Rago J, Tzagoloff A. The leader peptide of yeast Atp6p is required for efficient interaction with the Atp9p ring of the mitochondrial ATPase. J Biol Chem. 2007;282:36167-76 pubmed which the codons for amino acids 2-9 or 2-10 of the Atp6p precursor were deleted from the mitochondrial ATP6 gene. The concentration of Atp6p and ATPase complex was approximately 2 times less in the mutants...
  7. Michon T, Galante M, Velours J. NH2-terminal sequence of the isolated yeast ATP synthase subunit 6 reveals post-translational cleavage. Eur J Biochem. 1988;172:621-5 pubmed
    ..The amino acid composition of subunit 6 is close to the one predicted from the oli2 gene...
  8. Förster K, Turina P, Drepper F, Haehnel W, Fischer S, Graber P, et al. Proton transport coupled ATP synthesis by the purified yeast H+ -ATP synthase in proteoliposomes. Biochim Biophys Acta. 2010;1797:1828-37 pubmed publisher
    ..The dependence of the turnover on the phosphate concentration and the dependence of K(M) on pH(out) indicated that the substrate for ATP synthesis is the monoanionic phosphate species H?PO??. ..
  9. Velours J, Spannagel C, Chaignepain S, Vaillier J, Arselin G, Graves P, et al. Topography of the yeast ATP synthase F0 sector. Biochimie. 1998;80:793-801 pubmed
  10. Kucharczyk R, Salin B, di Rago J. 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. Hum Mol Genet. 2009;18:2889-98 pubmed publisher
    ..One of these mutations, T9176G, has been localized in the mitochondrial ATP6 gene encoding the Atp6p (or a) subunit of the ATP synthase...
  11. Murphy M, Roberts H, Choo W, Macreadie I, Marzuki S, Lukins H, et al. Biogenesis of mitochondria. oli2 Mutations affecting the coupling of oxidation to phosphorylation in Saccharomyces cerevisiae. Biochim Biophys Acta. 1980;592:431-44 pubmed
    ..1. Two oligomycin-resistant strains of Saccharomyces cerevisiae have been isolated and shown to have mutations in the oli2 region of the mitochondrial DNA...
  12. Paul M, Barrientos A, Tzagoloff A. A single amino acid change in subunit 6 of the yeast mitochondrial ATPase suppresses a null mutation in ATP10. J Biol Chem. 2000;275:29238-43 pubmed
    ..The suppressor mutation in the revertant has been mapped to the OLI2 locus in mitochondrial DNA and shown to be a single base change in the C-terminal coding region of the gene...
  13. Kabala A, Lasserre J, Ackerman S, di Rago J, Kucharczyk R. Defining the impact on yeast ATP synthase of two pathogenic human mitochondrial DNA mutations, T9185C and T9191C. Biochimie. 2014;100:200-6 pubmed publisher
    Mutations in the human mitochondrial ATP6 gene encoding ATP synthase subunit a/6 (referred to as Atp6p in yeast) are at the base of neurodegenerative disorders like Neurogenic Ataxia and Retinitis Pigmentosa (NARP), Leigh syndrome (LS), ..
  14. Bueler S, Rubinstein J. Location of subunit d in the peripheral stalk of the ATP synthase from Saccharomyces cerevisiae. Biochemistry. 2008;47:11804-10 pubmed publisher
    ..We propose that the C-terminal region of subunit d spans the gap from F 1 to F O, reinforcing this section of the peripheral stalk. ..
  15. Goyon V, Fronzes R, Salin B, di Rago J, Velours J, Brèthes D. Yeast cells depleted in Atp14p fail to assemble Atp6p within the ATP synthase and exhibit altered mitochondrial cristae morphology. J Biol Chem. 2008;283:9749-58 pubmed publisher
  16. Rubinstein J, Dickson V, Runswick M, Walker J. ATP synthase from Saccharomyces cerevisiae: location of subunit h in the peripheral stalk region. J Mol Biol. 2005;345:513-20 pubmed
    ..Models of the peripheral stalk are proposed that are consistent with this location and with reconstitution experiments conducted with isolated peripheral stalk subunits. ..
  17. Herrmann J, Stuart R, Craig E, Neupert W. Mitochondrial heat shock protein 70, a molecular chaperone for proteins encoded by mitochondrial DNA. J Cell Biol. 1994;127:893-902 pubmed
    ..Thus mt-Hsp70 by acting as a chaperone for proteins encoded by the mitochondrial DNA, has a critical role in the assembly of supra-molecular complexes. ..
  18. Zamudio Ochoa A, Camacho Villasana Y, García Guerrero A, Pérez Martínez X. The Pet309 pentatricopeptide repeat motifs mediate efficient binding to the mitochondrial COX1 transcript in yeast. RNA Biol. 2014;11:953-67 pubmed publisher
    ..This work demonstrates an in vivo physical interaction between a yeast mitochondrial translational activator and its target mRNA and shows the cooperativity of the PPR domains of Pet309 in interaction with the COX1 mRNA. ..
  19. Niedźwiecka K, Tisi R, Penna S, Lichocka M, Plochocka D, Kucharczyk R. Two mutations in mitochondrial ATP6 gene of ATP synthase, related to human cancer, affect ROS, calcium homeostasis and mitochondrial permeability transition in yeast. Biochim Biophys Acta Mol Cell Res. 2018;1865:117-131 pubmed publisher
    ..Here we show that two mutations, Atp6-P163S and Atp6-K90E (human MTATP6-P136S and MTATP6-K64E, found in prostate and thyroid cancer samples, respectively)..
  20. Spannagel C, Vaillier J, Chaignepain S, Velours J. Topography of the yeast ATP synthase F0 sector by using cysteine substitution mutants. Cross-linkings between subunits 4, 6, and f. Biochemistry. 1998;37:615-21 pubmed
  21. Horn D, Fontanesi F, Barrientos A. Exploring protein-protein interactions involving newly synthesized mitochondrial DNA-encoded proteins. Methods Mol Biol. 2008;457:125-39 pubmed
  22. Duvezin Caubet S, Rak M, Lefebvre Legendre L, Tetaud E, Bonnefoy N, di Rago J. A "petite obligate" mutant of Saccharomyces cerevisiae: functional mtDNA is lethal in cells lacking the delta subunit of mitochondrial F1-ATPase. J Biol Chem. 2006;281:16305-13 pubmed
    ..This is the first report of what we have called a "petite obligate" mutant of S. cerevisiae. ..
  23. Tzagoloff A, Barrientos A, Neupert W, Herrmann J. Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase. J Biol Chem. 2004;279:19775-80 pubmed
    ..Based on these observations, we propose Atp10p to be an Atp6p-specific chaperone that facilitates the incorporation of Atp6p into an intermediate subcomplex of ATPase subunits. ..
  24. Foury F, Roganti T, Lecrenier N, Purnelle B. The complete sequence of the mitochondrial genome of Saccharomyces cerevisiae. FEBS Lett. 1998;440:325-31 pubmed
    ..Our data suggest that shuffling of mobile elements plays an important role in the remodelling of the yeast mitochondrial genome. ..
  25. Rak M, Tzagoloff A. F1-dependent translation of mitochondrially encoded Atp6p and Atp8p subunits of yeast ATP synthase. Proc Natl Acad Sci U S A. 2009;106:18509-14 pubmed publisher
    ..of F(1), or the Atp11p and Atp12p chaperones that promote F(1) assembly, have normal levels of the bicistronic ATP8/ATP6 mRNAs but fail to synthesize Atp6p and Atp8p...
  26. Hell K, Neupert W, Stuart R. Oxa1p acts as a general membrane insertion machinery for proteins encoded by mitochondrial DNA. EMBO J. 2001;20:1281-8 pubmed
    ..Taken together, we conclude that the Oxa1 complex represents a general membrane protein insertion machinery in the inner membrane of mitochondria. ..
  27. Stephens A, Khan M, Roucou X, Nagley P, Devenish R. The molecular neighborhood of subunit 8 of yeast mitochondrial F1F0-ATP synthase probed by cysteine scanning mutagenesis and chemical modification. J Biol Chem. 2003;278:17867-75 pubmed
    ..We conclude that subunit 8 is an integral component of the stator stalk of yeast mitochondrial F(1)F(0)-ATP synthase. ..