Fumarylacetoacetic acid

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Fumarylacetoacetic acid
Fumarylacetoacetic acid.svg
Names
Preferred IUPAC name
(2E)-4,6-Dioxooct-2-enedioic acid
Identifiers
3D model (JSmol)
ChemSpider
MeSH Fumarylacetoacetate
PubChem CID
UNII
  • InChI=1S/C8H8O6/c9-5(1-2-7(11)12)3-6(10)4-8(13)14/h1-2H,3-4H2,(H,11,12)(H,13,14)/b2-1+
    Key: GACSIVHAIFQKTC-OWOJBTEDSA-N
  • InChI=1/C8H8O6/c9-5(1-2-7(11)12)3-6(10)4-8(13)14/h1-2H,3-4H2,(H,11,12)(H,13,14)/b2-1+
    Key: GACSIVHAIFQKTC-OWOJBTEDBU
  • O=C(\C=C\C(=O)O)CC(=O)CC(=O)O
Properties
C8H8O6
Molar mass 200.146 g·mol−1
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
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Fumarylacetoacetic acid (fumarylacetoacetate) is an intermediate in the metabolism of tyrosine. It is formed through the conversion of maleylacetoacetate into fumarylacetoacetate by the enzyme maleylacetoacetate isomerase. [1] Fumarylacetoacetate is hydrolyzed by the enzyme fumarylacetoacetate hydrolase (FAH), producing acetoacetate and fumarate. [2] These compounds may then enter various other metabolic pathways.

Fumarylacetoacetate may accumulate in patients with Tyrosinemia type I, in which there is a deficiency of the FAH enzyme. [3] In this disease, fumarylacetoacetate and precursors in the catabolism of tyrosine, including maleylacetoacetate, succinylacetone, and homogentisic acid.

See also

References

  1. ↑ W. E. Knox and M. LeMay-Knox (1951). "The oxidation in liver of l-tyrosine to acetoacetate through p-hydroxyphenylpyruvate and homogentisic acid". Biochem. J. 49 (5): 686–693. doi:10.1042/bj0490686. PMC   1197578 . PMID   14886367.
  2. ↑ Bateman, Raynard L.; Bhanumoorthy, P.; Witte, John F.; McClard, Ronald W.; Grompe, Markus; Timm, David E. (2001-01-01). "Mechanistic Inferences from the Crystal Structure of Fumarylacetoacetate Hydrolase with a Bound Phosphorus-based Inhibitor *". Journal of Biological Chemistry. 276 (18): 15284–15291. doi: 10.1074/jbc.M007621200 . ISSN   0021-9258. PMID   11154690.
  3. ↑ Chakrapani A, Holme E (2006). "Disorders of Tyrosine Metabolism". In Fernandes J, Saudubray JM, van den Berghe G, Walter JH (eds.). Inborn Metabolic Diseases. Springer. pp. 233–243. doi:10.1007/978-3-540-28785-8_18. ISBN   978-3-540-28785-8. PMC   1986449 .
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