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Gene Review

ACO2  -  aconitase 2, mitochondrial

Homo sapiens

Synonyms: ACONM, Aconitase, Aconitate hydratase, mitochondrial, Citrate hydro-lyase, ICRD
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Disease relevance of ACO2


High impact information on ACO2

  • After the action of the coenzyme B(12)-dependent 2-methyleneglutarate mutase and 3-methylitaconate isomerase, an aconitase and isocitrate lyase family pair of enzymes, (2R,3S)-dimethylmalate dehydratase and lyase, completes the pathway [5].
  • Furthermore, the refed rats show, in both SS and IMF muscle mitochondria, a lower aconitase activity (whose inactivation is an index of increased reactive oxygen species [ROS]), associated with higher superoxide dismutase activity and increased proton leak [6].
  • Activity of cytosolic xanthine oxidase, which holds a [2Fe-2S] cluster, was also specifically reduced, and iron-regulatory protein-1 was converted from its [4Fe-4S] aconitase form to its apo- or RNA-binding form [7].
  • Knock-downs of TbiscS and TbiscU in the procyclic stage by means of RNA interference resulted in reduced activity of the marker FeS enzyme aconitase in both the mitochondrion and cytosol because of the lack of FeS clusters [8].
  • IRP1 is a cytoplasmic aconitase when it contains a [4Fe-4S] cluster and an RNA-binding protein after complete removal of the metal center by an unknown mechanism [9].

Chemical compound and disease context of ACO2


Biological context of ACO2


Anatomical context of ACO2

  • These results show that a crayfish IRE, lacking the bulged C, can bind IRP1 in vitro and that an IRP1-like protein present in crayfish hepatopancreas may have both aconitase and RNA-binding activities [13].
  • In isolated mitochondria, Fe-S clusters were detected on newly imported yeast ferredoxin precursor and on endogenous aconitase by means of [35S]cysteine labeling and native gel separation [14].
  • BACKGROUND: Mitochondrial (m) aconitase plays an important role in the unique pathway of citrate accumulation in prostate epithelial cells through its limited activity [15].

Associations of ACO2 with chemical compounds


Other interactions of ACO2


Analytical, diagnostic and therapeutic context of ACO2


  1. Mutation and LOH analysis of ACO2 in colorectal cancer: no evidence of biallelic genetic inactivation. Laiho, P., Hienonen, T., Mecklin, J.P., Järvinen, H., Karhu, A., Launonen, V., Aaltonen, L.A. J. Med. Genet. (2003) [Pubmed]
  2. Voluntary breath-holding in the morning and in the evening. Bosco, G., Ionadi, A., Data, P.G., Mortola, J.P. Clin. Sci. (2004) [Pubmed]
  3. The rpfA gene of Xanthomonas campestris pathovar campestris, which is involved in the regulation of pathogenicity factor production, encodes an aconitase. Wilson, T.J., Bertrand, N., Tang, J.L., Feng, J.X., Pan, M.Q., Barber, C.E., Dow, J.M., Daniels, M.J. Mol. Microbiol. (1998) [Pubmed]
  4. Citrate enhances in vitro metastatic behaviours of PC-3M human prostate cancer cells: status of endogenous citrate and dependence on aconitase and fatty acid synthase. Mycielska, M.E., Broke-Smith, T.P., Palmer, C.P., Beckerman, R., Nastos, T., Erguler, K., Djamgoz, M.B. Int. J. Biochem. Cell Biol. (2006) [Pubmed]
  5. Molecular and functional analysis of nicotinate catabolism in Eubacterium barkeri. Alhapel, A., Darley, D.J., Wagener, N., Eckel, E., Elsner, N., Pierik, A.J. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  6. Altered skeletal muscle subsarcolemmal mitochondrial compartment during catch-up fat after caloric restriction. Crescenzo, R., Lionetti, L., Mollica, M.P., Ferraro, M., D'Andrea, E., Mainieri, D., Dulloo, A.G., Liverini, G., Iossa, S. Diabetes (2006) [Pubmed]
  7. RNA silencing of mitochondrial m-Nfs1 reduces Fe-S enzyme activity both in mitochondria and cytosol of mammalian cells. Fosset, C., Chauveau, M.J., Guillon, B., Canal, F., Drapier, J.C., Bouton, C. J. Biol. Chem. (2006) [Pubmed]
  8. Knock-downs of Iron-Sulfur Cluster Assembly Proteins IscS and IscU Down-regulate the Active Mitochondrion of Procyclic Trypanosoma brucei. Smíd, O., Horáková, E., Vilímová, V., Hrdy, I., Cammack, R., Horváth, A., Lukes, J., Tachezy, J. J. Biol. Chem. (2006) [Pubmed]
  9. Human cytoplasmic aconitase (Iron regulatory protein 1) is converted into its [3Fe-4S] form by hydrogen peroxide in vitro but is not activated for iron-responsive element binding. Brazzolotto, X., Gaillard, J., Pantopoulos, K., Hentze, M.W., Moulis, J.M. J. Biol. Chem. (1999) [Pubmed]
  10. Expression of 1-aminocyclopropane-1-carboxylate (ACC) oxidase genes during the development of vegetative tissues in white clover (Trifolium repens L.) is regulated by ontological cues. Chen, B.C., McManus, M.T. Plant Mol. Biol. (2006) [Pubmed]
  11. Regional localization of the genes coding for human ACO2, ARSA, and NAGA on chromosome 22. Geurts van Kessel, A.H., Westerveld, A., de Groot, P.G., Meera Khan, P., Hagemeijer, A. Cytogenet. Cell Genet. (1980) [Pubmed]
  12. Organization and structure of the 1-aminocyclopropane-1-carboxylate oxidase gene family from Petunia hybrida. Tang, X., Wang, H., Brandt, A.S., Woodson, W.R. Plant Mol. Biol. (1993) [Pubmed]
  13. An atypical iron-responsive element (IRE) within crayfish ferritin mRNA and an iron regulatory protein 1 (IRP1)-like protein from crayfish hepatopancreas. Huang, T.S., Melefors, O., Lind, M.I., Söderhäll, K. Insect Biochem. Mol. Biol. (1999) [Pubmed]
  14. Mrs3p, mrs4p, and frataxin provide iron for Fe-S cluster synthesis in mitochondria. Zhang, Y., Lyver, E.R., Knight, S.A., Pain, D., Lesuisse, E., Dancis, A. J. Biol. Chem. (2006) [Pubmed]
  15. Characterization of the mitochondrial aconitase promoter and the identification of transcription factor binding. Yu, Z., Costello, L.C., Feng, P., Franklin, R.B. Prostate (2006) [Pubmed]
  16. Peroxynitrite and nitric oxide differently target the iron-sulfur cluster and amino acid residues of human iron regulatory protein 1. Soum, E., Brazzolotto, X., Goussias, C., Bouton, C., Moulis, J.M., Mattioli, T.A., Drapier, J.C. Biochemistry (2003) [Pubmed]
  17. Prooxidant action of xanthurenic acid and quinoline compounds: role of transition metals in the generation of reactive oxygen species and enhanced formation of 8-hydroxy-2'-deoxyguanosine in DNA. Murakami, K., Haneda, M., Yoshino, M. Biometals (2006) [Pubmed]
  18. Differential accumulation of transcripts for ACC synthase and ACC oxidase homologs in etiolated mung bean hypocotyls in response to various stimuli. Yu, S.J., Kim, S., Lee, J.S., Lee, D.H. Mol. Cells (1998) [Pubmed]
  19. Expression of ACC oxidase genes differs among sex genotypes and sex phases in cucumber. Kahana, A., Silberstein, L., Kessler, N., Goldstein, R.S., Perl-Treves, R. Plant Mol. Biol. (1999) [Pubmed]
  20. Regional localization of the genes coding for human red cell adenylate kinase, aconitase, and galactose-1-phosphate uridylyltransferase on chromosome 9. Westerveld, A., Garver, J., Nijman, M.A., Pearson, P.L. Cytogenet. Cell Genet. (1978) [Pubmed]
  21. Aconitase (E.C. mitochondrial locus mapped to human chromosome 22: studies with Chinese hamster--human somatic cell hybrids. Sparkes, R.S., Mohandas, T., Sparkes, M.C., Shulkin, J.D. Biochem. Genet. (1978) [Pubmed]
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