Gene Symbol: Aco2
Description: aconitase 2
Alias: aconitate hydratase, mitochondrial, aconitase 2, mitochondrial, citrate hydro-lyase, mitochondrial aconitase (nuclear aco2 gene)
Species: rat
Products:     Aco2

Top Publications

  1. Chernyshev V, Mavlikaev R, Burtsev V, Dutko I, Zhidkov V. [Aerosol vaccination of broilers against Newcastle disease]. Veterinariia. 1978;:78-80 pubmed
  2. Bulteau A, Ikeda Saito M, Szweda L. Redox-dependent modulation of aconitase activity in intact mitochondria. Biochemistry. 2003;42:14846-55 pubmed
  3. Bulteau A, Lundberg K, Ikeda Saito M, Isaya G, Szweda L. Reversible redox-dependent modulation of mitochondrial aconitase and proteolytic activity during in vivo cardiac ischemia/reperfusion. Proc Natl Acad Sci U S A. 2005;102:5987-91 pubmed
    ..Thus, the response of mitochondrial aconitase and ATP-dependent proteolytic activity to reperfusion-induced prooxidant production appears to be a regulated event that would be expected to reduce irreparable damage to the mitochondria. ..
  4. Oktay Y, Dioum E, Matsuzaki S, Ding K, Yan L, Haller R, et al. Hypoxia-inducible factor 2alpha regulates expression of the mitochondrial aconitase chaperone protein frataxin. J Biol Chem. 2007;282:11750-6 pubmed
    ..These findings expand upon the previously defined role of HIF-2alpha in the cellular response to oxidative stress and identify a novel link of HIF-2alpha with mitochondrial homeostasis. ..
  5. Masini A, Guarriero Bobyleva V, Pincelli Gatti A, Cennamo C. Developmental changes of rat liver cytoplasmic and mitochondrial aconitate hydratases. Ital J Biochem. 1978;27:300-4 pubmed
    ..The developmental pattern of the activity of the cytoplasmic aconitate hydratase may suggest a possible role of this enzyme in gluconeogenesis. ..
  6. Mirel D, Marder K, Graziano J, Freyer G, Zhao Q, Mayeux R, et al. Characterization of the human mitochondrial aconitase gene (ACO2). Gene. 1998;213:205-18 pubmed
    We have cloned and characterized the ACO2 gene on human chromosome 22q13, which encodes the essential iron-dependent metabolic enzyme mitochondrial aconitase...
  7. Eprintsev A, Semenova E, Popov V. Induction of aconitate hydratase in hepatocytes of starving rats. Biochemistry (Mosc). 2002;67:795-801 pubmed
    ..The purified preparations of these AH isoforms were electrophoretically homogenous. The molecular weights of these isoforms were estimated and several kinetic and regulatory properties were studied. ..
  8. 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. ..
  9. Muller F, Liu Y, Van Remmen H. Complex III releases superoxide to both sides of the inner mitochondrial membrane. J Biol Chem. 2004;279:49064-73 pubmed
    ..This explains extramitochondrial release of superoxide but raises the question of how superoxide could reach the matrix. We discuss two models explaining this result...

More Information


  1. Ilangovan G, Venkatakrishnan C, Bratasz A, Osinbowale S, Cardounel A, Zweier J, et al. Heat shock-induced attenuation of hydroxyl radical generation and mitochondrial aconitase activity in cardiac H9c2 cells. Am J Physiol Cell Physiol. 2006;290:C313-24 pubmed
    ..In conclusion, we propose that attenuation of *OH generation after heat shock treatment might play an important role in reducing the myocardial ischemic injury, observed in heat shock-treated animals. ..
  2. Lin G, Brownsey R, MacLeod K. Regulation of mitochondrial aconitase by phosphorylation in diabetic rat heart. Cell Mol Life Sci. 2009;66:919-32 pubmed publisher
    ..This may influence the activity of the tricarboxylic acid cycle, and contribute to impaired mitochondrial function and energy metabolism in diabetic hearts. ..
  3. Slaughter C, Hopkinson D, Harris H. Aconitase polymorphism in man. Ann Hum Genet. 1975;39:193-202 pubmed
    ..The variation due to the three most common ACONS alleles in the Nigerians constitues a polymorphism. ..
  4. Andersson U, Leighton B, Young M, Blomstrand E, Newsholme E. Inactivation of aconitase and oxoglutarate dehydrogenase in skeletal muscle in vitro by superoxide anions and/or nitric oxide. Biochem Biophys Res Commun. 1998;249:512-6 pubmed
    ..It is suggested that these effects may be responsible for some alterations in skeletal muscle metabolism, and these possibilities are discussed. ..
  5. Kim S, Marekov L, Bubber P, Browne S, Stavrovskaya I, Lee J, et al. Mitochondrial aconitase is a transglutaminase 2 substrate: transglutamination is a probable mechanism contributing to high-molecular-weight aggregates of aconitase and loss of aconitase activity in Huntington disease brain. Neurochem Res. 2005;30:1245-55 pubmed
    ..The present findings suggest that an increase of transglutaminase activity in HD caudate may contribute to mitochondrial dysfunction by incorporating aconitase into inactive polymers. ..
  6. Zhuravliova E, Barbakadze T, Zaalishvili E, Chipashvili M, Koshoridze N, Mikeladze D. Social isolation in rats inhibits oxidative metabolism, decreases the content of mitochondrial K-Ras and activates mitochondrial hexokinase. Behav Brain Res. 2009;205:377-83 pubmed publisher
  7. Kim S, Cheresh P, Williams D, Cheng Y, Ridge K, Schumacker P, et al. Mitochondria-targeted Ogg1 and aconitase-2 prevent oxidant-induced mitochondrial DNA damage in alveolar epithelial cells. J Biol Chem. 2014;289:6165-76 pubmed publisher
    ..Furthermore, mt-hOgg1 chaperoning of Aco-2 in preventing oxidant-mediated mtDNA damage and apoptosis may afford an innovative target for the molecular events underlying oxidant-induced toxicity...