Fumarylacetoacetic acid
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| Preferred IUPAC name
(2E)-4,6-Dioxooct-2-enedioic acid | |
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3D model (JSmol)
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| ChemSpider | |
| MeSH | Fumarylacetoacetate |
PubChem CID
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CompTox Dashboard (EPA)
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| 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
- ^ 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.