4527 4-Fumarylacetoacetic acid Fumarylacetoacetate is an intermediate in the metabolism of tyrosine. Fumarylacetoacetate hydrolase (FAH) is an enzyme which catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. FAH is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia. 28613-33-4 5280398 C8H8O6 White powder. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. 2014-08-29T06:51:36Z 2026-04-06T11:09:47Z Fumarylacetoacetate C01061 30907 4-FUMARYL-ACETOACETATE true OC(=O)CC(=O)CC(=O)\C=C\C(O)=O C8H8O6 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+ GACSIVHAIFQKTC-OWOJBTEDSA-N 200.1455 200.032087988 Endogenous Solid HMDB01268 4444081 CHEM003433 NS00121523 (2Z)-4,6-dioxooct-2-enedioic acid