Fumarylacetoacetic acid

Fumarylacetoacetic acid
Names
	Preferred IUPAC name (2E)-4,6-Dioxooct-2-enedioic acid
Identifiers
CAS Number	28613-33-4 ; 5698-51-1 ;
3D model (JSmol)	Interactive image;
ChemSpider	4444081;
MeSH	Fumarylacetoacetate
PubChem CID	5280398;
UNII	FM3BV7K438 ;
CompTox Dashboard (EPA)	DTXSID20901388 ;
	InChI 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;
	SMILES O=C(\C=C\C(=O)O)CC(=O)CC(=O)O;
Properties
Chemical formula	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). verify (what is ?) Infobox references

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.

References

^ 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.
^ 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.
^ 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.

[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.

[Chakrapani_2006-3] 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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[2]

[3]

See also

References