Gene Symbol: ACO1
Description: aconitate hydratase ACO1
Alias: GLU1, aconitate hydratase ACO1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Chen X, Wang X, Kaufman B, Butow R. Aconitase couples metabolic regulation to mitochondrial DNA maintenance. Science. 2005;307:714-7 pubmed
    ..Regulation of ACO1 expression by the HAP and retrograde metabolic signaling pathways directly affects mtDNA maintenance...
  2. Chen X, Wang X, Butow R. Yeast aconitase binds and provides metabolically coupled protection to mitochondrial DNA. Proc Natl Acad Sci U S A. 2007;104:13738-43 pubmed
    ..Therefore, mitochondria are opportunistic organelles that seize proteins, such as metabolic enzymes, for construction of the nucleoid, an mtDNA maintenance/segregation apparatus. ..
  3. Lin A, Hakala K, Weintraub S, McAlister Henn L. Suppression of metabolic defects of yeast isocitrate dehydrogenase and aconitase mutants by loss of citrate synthase. Arch Biochem Biophys. 2008;474:205-12 pubmed publisher
  4. Gangloff S, Marguet D, Lauquin G. Molecular cloning of the yeast mitochondrial aconitase gene (ACO1) and evidence of a synergistic regulation of expression by glucose plus glutamate. Mol Cell Biol. 1990;10:3551-61 pubmed
    We have isolated genomic clones complementing the aconitase-deficient strain (glu1-1) of Saccharomyces cerevisiae. Identification of the aconitase gene was established by enzymatic assays and molecular analyses...
  5. Kingsbury J, Sen N, Cardenas M. Branched-Chain Aminotransferases Control TORC1 Signaling in Saccharomyces cerevisiae. PLoS Genet. 2015;11:e1005714 pubmed publisher
  6. Rezaei M, Aslankoohi E, Verstrepen K, Courtin C. Contribution of the tricarboxylic acid (TCA) cycle and the glyoxylate shunt in Saccharomyces cerevisiae to succinic acid production during dough fermentation. Int J Food Microbiol. 2015;204:24-32 pubmed publisher
    ..More specifically, deletion of ACO1 and double deletion of ACO1 and ICL1 resulted in a 36 and 77% decrease in succinic acid levels in fermented dough, ..
  7. Fazius F, Shelest E, Gebhardt P, Brock M. The fungal ?-aminoadipate pathway for lysine biosynthesis requires two enzymes of the aconitase family for the isomerization of homocitrate to homoisocitrate. Mol Microbiol. 2012;86:1508-30 pubmed publisher
  8. Melber A, Na U, Vashisht A, Weiler B, Lill R, Wohlschlegel J, et al. Role of Nfu1 and Bol3 in iron-sulfur cluster transfer to mitochondrial clients. elife. 2016;5: pubmed publisher
    ..Given these observations, we propose that Nfu1, assisted by Bol3, functions to facilitate Fe-S transfer from the biosynthetic apparatus to the client proteins preventing oxidative damage to [4Fe-4S] clusters. ..
  9. Kaufman B, Newman S, Hallberg R, Slaughter C, Perlman P, Butow R. In organello formaldehyde crosslinking of proteins to mtDNA: identification of bifunctional proteins. Proc Natl Acad Sci U S A. 2000;97:7772-7 pubmed
    ..These data identify bifunctional proteins that participate in the stability of rho(+) mtDNA. ..

More Information


  1. Bareth B, Nikolov M, Lorenzi I, Hildenbeutel M, Mick D, Helbig C, et al. Oms1 associates with cytochrome c oxidase assembly intermediates to stabilize newly synthesized Cox1. Mol Biol Cell. 2016;27:1570-80 pubmed publisher
    ..Our analyses show that Oms1 participates in cytochrome c oxidase assembly by stabilizing newly synthesized Cox1. ..
  2. Kumar A, Bachhawat A. OXP1/YKL215c encodes an ATP-dependent 5-oxoprolinase in Saccharomyces cerevisiae: functional characterization, domain structure and identification of actin-like ATP-binding motifs in eukaryotic 5-oxoprolinases. FEMS Yeast Res. 2010;10:394-401 pubmed publisher
    ..The results are discussed in the light of the previously postulated truncated gamma-glutamyl cycle of yeasts. ..
  3. Kucej M, Kucejova B, SUBRAMANIAN R, Chen X, Butow R. Mitochondrial nucleoids undergo remodeling in response to metabolic cues. J Cell Sci. 2008;121:1861-8 pubmed publisher
    ..A mutant form of Hsp60 that causes mtDNA instability has altered submitochondrial localization, which suggests that nucleoid remodeling is essential for the maintenance of mitochondrial genome...
  4. Guirola M, Jimenez Marti E, Atrian S. On the molecular relationships between high-zinc tolerance and aconitase (Aco1) in Saccharomyces cerevisiae. Metallomics. 2014;6:634-45 pubmed publisher
    ..redistribution of the aconitase protein to mitochondria, and also a loss of aconitase activity, despite normal Aco1 protein levels being present, probably due to the epistasis in protecting mtDNA between PIF1 and ACO1...
  5. Farooq M, Pracheil T, Dong Z, Xiao F, Liu Z. Mitochondrial DNA instability in cells lacking aconitase correlates with iron citrate toxicity. Oxid Med Cell Longev. 2013;2013:493536 pubmed publisher
    Aconitase, the second enzyme of the tricarboxylic acid cycle encoded by ACO1 in the budding yeast Saccharomyces cerevisiae, catalyzes the conversion of citrate to isocitrate. aco1? results in mitochondrial DNA (mtDNA) instability...
  6. James A, Cocheme H, Murai M, Miyoshi H, Murphy M. Complementation of coenzyme Q-deficient yeast by coenzyme Q analogues requires the isoprenoid side chain. FEBS J. 2010;277:2067-82 pubmed publisher
    ..Here we suggest that CoQ or its redox state may be a signal for growth during the shift to respiration. ..
  7. Vögtle F, Wortelkamp S, Zahedi R, Becker D, Leidhold C, Gevaert K, et al. Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability. Cell. 2009;139:428-39 pubmed publisher
    ..Our results suggest that Icp55 is critical for stabilization of the mitochondrial proteome and illustrate how the N-proteome can serve as rich source for a systematic analysis of mitochondrial protein targeting, cleavage and turnover. ..
  8. Regev Rudzki N, Karniely S, Ben Haim N, Pines O. Yeast aconitase in two locations and two metabolic pathways: seeing small amounts is believing. Mol Biol Cell. 2005;16:4163-71 pubmed
    ..the beta-galactosidase enzyme to aconitase and observed alpha complementation in the cytosol; and 2) we created an ACO1-URA3 hybrid gene, which allowed isolation of strains in which the hybrid protein is exclusively targeted to ..
  9. Bulteau A, O Neill H, Kennedy M, Ikeda Saito M, Isaya G, Szweda L. Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity. Science. 2004;305:242-5 pubmed
    ..Thus, frataxin is an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation. ..
  10. McAlister Henn L, Small W. Molecular genetics of yeast TCA cycle isozymes. Prog Nucleic Acid Res Mol Biol. 1997;57:317-39 pubmed
  11. Puig S, Askeland E, Thiele D. Coordinated remodeling of cellular metabolism during iron deficiency through targeted mRNA degradation. Cell. 2005;120:99-110 pubmed
  12. Epstein C, Waddle J, Hale W, Dave V, Thornton J, Macatee T, et al. Genome-wide responses to mitochondrial dysfunction. Mol Biol Cell. 2001;12:297-308 pubmed
    ..Transcript profiling of cells harboring null alleles of RTG1, RTG2, or RTG3, genes known to control signaling from mitochondria to the nucleus, suggests that there are multiple pathways of cross-talk between these organelles in yeast. ..