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Chemical Compound Review

AG-G-00707     4,6-dioxooct-2-enedioic acid

Synonyms: AG-K-88794, CTK5A6109, CTK7G0822, AC1L18J0, 5698-52-2, ...
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Disease relevance of C01036


High impact information on C01036


Biological context of C01036


Anatomical context of C01036


Associations of C01036 with other chemical compounds


Gene context of C01036

  • FAA treatment also causes a subsequent induction of the proapoptotic CHOP (CEBP homologous protein) transcription factor as well as a late activation of caspase-12 [8].
  • Tyrosinaemia type 1 and glutathione synthetase deficiency: two disorders with reduced hepatic thiol group concentrations and a liver 4-fumarylacetoacetate hydrolase deficiency [6].

Analytical, diagnostic and therapeutic context of C01036


  1. Hepatocyte injury in tyrosinemia type 1 is induced by fumarylacetoacetate and is inhibited by caspase inhibitors. Kubo, S., Sun, M., Miyahara, M., Umeyama, K., Urakami, K., Yamamoto, T., Jakobs, C., Matsuda, I., Endo, F. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  2. Cloning and expression of the cDNA encoding human fumarylacetoacetate hydrolase, the enzyme deficient in hereditary tyrosinemia: assignment of the gene to chromosome 15. Phaneuf, D., Labelle, Y., Bérubé, D., Arden, K., Cavenee, W., Gagné, R., Tanguay, R.M. Am. J. Hum. Genet. (1991) [Pubmed]
  3. Fumarylacetoacetate, the metabolite accumulating in hereditary tyrosinemia, activates the ERK pathway and induces mitotic abnormalities and genomic instability. Jorquera, R., Tanguay, R.M. Hum. Mol. Genet. (2001) [Pubmed]
  4. Cyclin B-dependent kinase and caspase-1 activation precedes mitochondrial dysfunction in fumarylacetoacetate-induced apoptosis. Jorquera, R., Tanguay, R.M. FASEB J. (1999) [Pubmed]
  5. Mass spectral characterization of dichloroacetic acid-modified human glutathione transferase zeta. Anderson, W.B., Liebler, D.C., Board, P.G., Anders, M.W. Chem. Res. Toxicol. (2002) [Pubmed]
  6. Tyrosinaemia type 1 and glutathione synthetase deficiency: two disorders with reduced hepatic thiol group concentrations and a liver 4-fumarylacetoacetate hydrolase deficiency. Lloyd, A.J., Gray, R.G., Green, A. J. Inherit. Metab. Dis. (1995) [Pubmed]
  7. Characterization and substrate specificity of fumarylacetoacetate fumarylhydrolase. Mahuran, D.J., Angus, R.H., Braun, C.V., Sim, S.S., Schmidt, D.E. Can. J. Biochem. (1977) [Pubmed]
  8. Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I. Bergeron, A., Jorquera, R., Orejuela, D., Tanguay, R.M. J. Biol. Chem. (2006) [Pubmed]
  9. Deficient fumarylacetoacetate fumarylhydrolase activity in lymphocytes and fibroblasts from patients with hereditary tyrosinemia. Kvittingen, E.A., Halvorsen, S., Jellum, E. Pediatr. Res. (1983) [Pubmed]
  10. The structure of a zeta class glutathione S-transferase from Arabidopsis thaliana: characterisation of a GST with novel active-site architecture and a putative role in tyrosine catabolism. Thom, R., Dixon, D.P., Edwards, R., Cole, D.J., Lapthorn, A.J. J. Mol. Biol. (2001) [Pubmed]
  11. Alkylation and inactivation of human glutathione transferase zeta (hGSTZ1-1) by maleylacetone and fumarylacetone. Lantum, H.B., Liebler, D.C., Board, P.G., Anders, M.W. Chem. Res. Toxicol. (2002) [Pubmed]
  12. Crystal structure and mechanism of a carbon-carbon bond hydrolase. Timm, D.E., Mueller, H.A., Bhanumoorthy, P., Harp, J.M., Bunick, G.J. Structure (1999) [Pubmed]
  13. Emergent mechanistic diversity of enzyme-catalysed beta-diketone cleavage. Grogan, G. Biochem. J. (2005) [Pubmed]
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