Gene Symbol: Acon
Description: Aconitase
Alias: ACON-1, AconM, BEST:GH10550, CG9244, Dmel\CG9244, FBgn0010100, M-Acon, acon, hsp60, i204, i59, l(2)07054, m-Acon, m-acon, mACON, mAc, mAcon, aconitase, ACONITASE, Acon-PB, Acon-PD, Acon-PE, Acon-PF, CG9244-PB, CG9244-PD, CG9244-PE, CG9244-PF, m-aconitase, mitochondrial aconitase
Species: fruit fly

Top Publications

  1. Kirby K, Hu J, Hilliker A, Phillips J. RNA interference-mediated silencing of Sod2 in Drosophila leads to early adult-onset mortality and elevated endogenous oxidative stress. Proc Natl Acad Sci U S A. 2002;99:16162-7 pubmed
  2. Missirlis F, Hu J, Kirby K, Hilliker A, Rouault T, Phillips J. Compartment-specific protection of iron-sulfur proteins by superoxide dismutase. J Biol Chem. 2003;278:47365-9 pubmed
    ..Conversely, mitochondrial aconitase activity is relatively insensitive to loss of Sod1 function, but drops dramatically if Sod2 activity is ..
  3. Anderson P, Kirby K, Hilliker A, Phillips J. RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila. Hum Mol Genet. 2005;14:3397-405 pubmed
  4. Fergestad T, Bostwick B, Ganetzky B. Metabolic disruption in Drosophila bang-sensitive seizure mutants. Genetics. 2006;173:1357-64 pubmed
    ..Our data implicate cellular metabolism in regulating seizure susceptibility and suggest that differential sensitivity of neuronal subtypes to metabolic changes underlies distinct types of seizure activity. ..
  5. Das N, Levine R, Orr W, Sohal R. Selectivity of protein oxidative damage during aging in Drosophila melanogaster. Biochem J. 2001;360:209-16 pubmed
    ..b>Aconitase was found to be the only protein in the mitochondrial matrix that exhibited an age-associated increase in ..
  6. Bayat V, Thiffault I, Jaiswal M, Tetreault M, Donti T, Sasarman F, et al. Mutations in the mitochondrial methionyl-tRNA synthetase cause a neurodegenerative phenotype in flies and a recessive ataxia (ARSAL) in humans. PLoS Biol. 2012;10:e1001288 pubmed publisher
    ..Patient cells also exhibited reduced Complex I activity, increased ROS, and a slower cell proliferation rate, similar to Drosophila Aats-met mutants...
  7. Domanitskaya E, Liu H, Chen S, Kubli E. The hydroxyproline motif of male sex peptide elicits the innate immune response in Drosophila females. FEBS J. 2007;274:5659-68 pubmed
    ..We suggest that SP acts by chemical mimicry of sugar components of the bacterial cell wall. Thus, SP may induce the immune system via pattern recognition receptors. ..
  8. Kirby K, Jensen L, Binnington J, Hilliker A, Ulloa J, Culotta V, et al. Instability of superoxide dismutase 1 of Drosophila in mutants deficient for its cognate copper chaperone. J Biol Chem. 2008;283:35393-401 pubmed publisher
    ..mutants in terms of reduced adult life span, hypersensitivity to oxidative stress, and loss of cytosolic aconitase activity...
  9. Sen A, Damm V, Cox R. Drosophila clueless is highly expressed in larval neuroblasts, affects mitochondrial localization and suppresses mitochondrial oxidative damage. PLoS ONE. 2013;8:e54283 pubmed publisher
    ..Thus Clu's role in mitochondrial function is not critical during larval development, but is important for pupae and adults. ..

More Information


  1. Abramov Y, Shatskikh A, Maksimenko O, Bonaccorsi S, Gvozdev V, Lavrov S. The Differences Between Cis- and Trans-Gene Inactivation Caused by Heterochromatin in Drosophila. Genetics. 2016;202:93-106 pubmed publisher
    ..We demonstrate that trans-inactivation is followed by de novo HP1a accumulation in the affected transgene; trans-inactivation is specifically favored by the chromatin remodeler SAYP and prevented by Argonaute AGO2. ..
  2. Chandra S, Pandey A, Chowdhuri D. MiRNA profiling provides insights on adverse effects of Cr(VI) in the midgut tissues of Drosophila melanogaster. J Hazard Mater. 2015;283:558-67 pubmed publisher
    ..Down-regulation of mus309 and mus312 under DNA repair, acon to oxidation-reduction and pyd to stress activated MAPK cascade respectively belonging to these gene ontology ..
  3. Tian L, Chen J, Chen M, Gui C, Zhong C, Hong L, et al. The p38 pathway regulates oxidative stress tolerance by phosphorylation of mitochondrial protein IscU. J Biol Chem. 2014;289:31856-65 pubmed publisher
    ..These results imply a new mechanistic connection between the p38 pathway and mitochondria iron-sulfur clusters. ..
  4. Riebler A, Held L, Stephan W. Bayesian variable selection for detecting adaptive genomic differences among populations. Genetics. 2008;178:1817-29 pubmed publisher
    ..For data sets with small sample sizes, high mutation rates, and/or long sequences, however, methods based on nucleotide statistics should be preferred. ..
  5. Lindsay H, Baines R, ffrench Constant R, Lilley K, Jacobs H, O Dell K. The dominant cold-sensitive Out-cold mutants of Drosophila melanogaster have novel missense mutations in the voltage-gated sodium channel gene paralytic. Genetics. 2008;180:873-84 pubmed publisher
    ..Therefore, in the absence of suitable vertebrate models, Ocd provides a system in which genetic, molecular, physiological, and behavioral tools can be exploited to determine mechanisms underlying sodium channel periodic paralyses...
  6. Esposito G, Vos M, Vilain S, Swerts J, De Sousa Valadas J, Van Meensel S, et al. Aconitase causes iron toxicity in Drosophila pink1 mutants. PLoS Genet. 2013;9:e1003478 pubmed publisher
    ..the molecular mechanisms of pink1 function, we performed a genetic modifier screen in Drosophila and identified aconitase (acon) as a dominant suppressor of pink1...
  7. Qin Q, Wang X, Zhou B. Functional studies of Drosophila zinc transporters reveal the mechanism for dietary zinc absorption and regulation. BMC Biol. 2013;11:101 pubmed publisher
    ..The knowledge gained will act as a reference for future mammalian studies, and also enable an appreciation of this important process from an evolutionary perspective. ..
  8. Zhang K, Li Z, Jaiswal M, Bayat V, Xiong B, Sandoval H, et al. The C8ORF38 homologue Sicily is a cytosolic chaperone for a mitochondrial complex I subunit. J Cell Biol. 2013;200:807-20 pubmed publisher
    ..Our data indicate that cytosolic chaperones are required for the subcellular transport of ND42. ..
  9. Lim D, Lee L, Oh C, Kim N, Hwang S, Han S, et al. Microarray analysis of Drosophila dicer-2 mutants reveals potential regulation of mitochondrial metabolism by endogenous siRNAs. J Cell Biochem. 2013;114:418-27 pubmed publisher
    ..Our data support a potential role for the endo-siRNA pathway in energy homeostasis through regulation of mitochondrial metabolism. ..
  10. Lozinsky O, Lushchak O, Kryshchuk N, Shchypanska N, Riabkina A, Skarbek S, et al. S-nitrosoglutathione-induced toxicity in Drosophila melanogaster: Delayed pupation and induced mild oxidative/nitrosative stress in eclosed flies. Comp Biochem Physiol A Mol Integr Physiol. 2013;164:162-70 pubmed publisher
    ..Larval exposure to GSNO resulted in lower activities of aconitase in both sexes and also lower activities of catalase and isocitrate dehydrogenase in adult males relative to the ..
  11. Scialò F, Sriram A, Fernández Ayala D, Gubina N, Lõhmus M, Nelson G, et al. Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan. Cell Metab. 2016;23:725-34 pubmed publisher
    ..These results illustrate that the source of a ROS signal is vital in determining its effects on cellular physiology and establish that manipulation of ubiquinone redox state is a valid strategy to delay aging. ..
  12. Celotto A, Chiu W, Van Voorhies W, Palladino M. Modes of metabolic compensation during mitochondrial disease using the Drosophila model of ATP6 dysfunction. PLoS ONE. 2011;6:e25823 pubmed publisher
    ..These studies demonstrate the dynamic nature of metabolic compensatory mechanisms and emphasize the need for time course studies in tractable animal systems to elucidate disease pathogenesis and novel therapeutic avenues. ..
  13. Wu Z, Du Y, Xue H, Wu Y, Zhou B. Aluminum induces neurodegeneration and its toxicity arises from increased iron accumulation and reactive oxygen species (ROS) production. Neurobiol Aging. 2012;33:199.e1-12 pubmed publisher
    ..Our results indicate that Al toxicity is mediated through ROS production and iron accumulation and suggest a remedial route to reduce toxicity due to Al exposure. ..
  14. Paul A, Belton A, Nag S, Martin I, Grotewiel M, Duttaroy A. Reduced mitochondrial SOD displays mortality characteristics reminiscent of natural aging. Mech Ageing Dev. 2007;128:706-16 pubmed
    ..b>Mitochondrial aconitase activity was substantially reduced in the Sod2 mutants, suggesting that SOD2 normally ensures the ..
  15. Jordan K, Morgan T, Mackay T. Quantitative trait loci for locomotor behavior in Drosophila melanogaster. Genetics. 2006;174:271-84 pubmed
    ..These data implicate variation in the synthesis of bioamines as a factor contributing to natural variation in locomotor reactivity. ..
  16. Sun X, Komatsu T, Lim J, Laslo M, Yolitz J, Wang C, et al. Nutrient-dependent requirement for SOD1 in lifespan extension by protein restriction in Drosophila melanogaster. Aging Cell. 2012;11:783-93 pubmed publisher
  17. Kanda H, Igaki T, Okano H, Miura M. Conserved metabolic energy production pathways govern Eiger/TNF-induced nonapoptotic cell death. Proc Natl Acad Sci U S A. 2011;108:18977-82 pubmed publisher
    ..This result suggests a link between sensitivity to cell death and metabolic activity in cancer. ..
  18. Wicks S, Bain N, Duttaroy A, Hilliker A, Phillips J. Hypoxia rescues early mortality conferred by superoxide dismutase deficiency. Free Radic Biol Med. 2009;46:176-81 pubmed publisher
    ..Elucidating these pathways should provide novel insights into how aerobic cells manage oxidative stress in health, aging, and disease. ..
  19. Tricoire H, Palandri A, Bourdais A, Camadro J, Monnier V. Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia. Hum Mol Genet. 2014;23:968-79 pubmed publisher
    ..This work provides the grounds for further evaluation of MB action in mammals. ..
  20. Llorens J, Navarro J, Martínez Sebastián M, Baylies M, Schneuwly S, Botella J, et al. Causative role of oxidative stress in a Drosophila model of Friedreich ataxia. FASEB J. 2007;21:333-44 pubmed
    ..Remarkably, frataxin overexpression also induced the oxidative-mediated inactivation of mitochondrial aconitase. This work demonstrates, for the first time, the essential function of frataxin in protecting aconitase ..
  21. Terhzaz S, Southall T, Lilley K, Kean L, Allan A, Davies S, et al. Differential gel electrophoresis and transgenic mitochondrial calcium reporters demonstrate spatiotemporal filtering in calcium control of mitochondria. J Biol Chem. 2006;281:18849-58 pubmed
    ..The results highlight the dynamic nature and both spatial and temporal heterogeneity of calcium signaling possible in differentiated, organotypic cells and provide a new model for neuroendocrine control of V-ATPase. ..
  22. McGurk L, Bonini N. Protein interacting with C kinase (PICK1) is a suppressor of spinocerebellar ataxia 3-associated neurodegeneration in Drosophila. Hum Mol Genet. 2012;21:76-84 pubmed publisher
    ..These findings indicate that interaction proteins may define a rich source of modifier pathways to target in disease situations. ..
  23. Cheng Z, Tsuda M, Kishita Y, Sato Y, Aigaki T. Impaired energy metabolism in a Drosophila model of mitochondrial aconitase deficiency. Biochem Biophys Res Commun. 2013;433:145-50 pubmed publisher
    ..Here we investigated the effect of gene knockout and knockdown of the mitochondrial aconitase Acon in Drosophila...
  24. Navarro J, Botella J, Metzendorf C, Lind M, Schneuwly S. Mitoferrin modulates iron toxicity in a Drosophila model of Friedreich's ataxia. Free Radic Biol Med. 2015;85:71-82 pubmed publisher
  25. Dorus S, Freeman Z, Parker E, Heath B, Karr T. Recent origins of sperm genes in Drosophila. Mol Biol Evol. 2008;25:2157-66 pubmed publisher
    ..Thus, gene creation is a dynamic force in the evolution of sperm composition and possibly function, which further suggests that acquisition of molecular functionality in sperm may be an influential pathway in the fixation of new genes. ..