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Gene Review

ACO2  -  aconitase 2, mitochondrial

Sus scrofa

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Disease relevance of ACO2


High impact information on ACO2


Anatomical context of ACO2

  • 19F NMR establishes that rat and mouse liver cytosols defluorinate (-)- but not (+)-erythro-fluorocitrate and pig heart aconitase also defluorinates (-)-erythro-fluorocitrate.(ABSTRACT TRUNCATED AT 250 WORDS)[6]

Analytical, diagnostic and therapeutic context of ACO2

  • Three of these four cysteines (peptides 3 and 7) correlated with those proposed to be cluster ligands recently determined by x-ray crystallography (Robbins, A. H. and Stout, C. D. (1989) Proteins, in press; Robbins, A. H., and Stout, C. D.,, (1989) Proc. Natl. Acad. Sci. U. S. A. 86, 3639-3643) for pig heart aconitase [4].


  1. Mutational analysis of active site residues in pig heart aconitase. Zheng, L., Kennedy, M.C., Beinert, H., Zalkin, H. J. Biol. Chem. (1992) [Pubmed]
  2. Structure of activated aconitase: formation of the [4Fe-4S] cluster in the crystal. Robbins, A.H., Stout, C.D. Proc. Natl. Acad. Sci. U.S.A. (1989) [Pubmed]
  3. Cloning and structural characterization of porcine heart aconitase. Zheng, L., Andrews, P.C., Hermodson, M.A., Dixon, J.E., Zalkin, H. J. Biol. Chem. (1990) [Pubmed]
  4. Cysteine labeling studies of beef heart aconitase containing a 4Fe, a cubane 3Fe, or a linear 3Fe cluster. Plank, D.W., Kennedy, M.C., Beinert, H., Howard, J.B. J. Biol. Chem. (1989) [Pubmed]
  5. Identification of the reactive sulfhydryl and sequences of cysteinyl-tryptic peptides from beef heart aconitase. Plank, D.W., Howard, J.B. J. Biol. Chem. (1988) [Pubmed]
  6. Enzymatic defluorination and metabolism of fluoroacetate, fluoroacetamide, fluoroethanol, and (-)-erythro-fluorocitrate in rats and mice examined by 19F and 13C NMR. Tecle, B., Casida, J.E. Chem. Res. Toxicol. (1989) [Pubmed]
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