4-Fumarylacetoacetic acid (CHEM003433)

Record Information
Version1.0
Creation Date2014-08-29 06:51:36 UTC
Update Date2026-04-06 11:09:47 UTC
Accession NumberCHEM003433
Identification
Common Name4-Fumarylacetoacetic acid
ClassSmall Molecule
Description
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.
Contaminant Sources
  • FooDB Chemicals
  • T3DB toxins
Contaminant Type
  • Animal Toxin
  • Food Toxin
  • Metabolite
  • Natural Compound
  • Organic Compound
Chemical Structure
MOLSDF3D SDFPDBSMILESInChI View 3D Structure
Synonyms
ValueSource
4-MaleylacetoacetateChEBI
4-Maleylacetoacetic acidKegg
MaleylacetoacetateGenerator
(2Z)-4,6-Dioxo-2-octenedioic acidHMDB
Maleylacetoacetic acidHMDB
Chemical FormulaC8H8O6
Average Molecular Mass200.146 g/mol
Monoisotopic Mass200.032 g/mol
CAS Registry Number28613-33-4
IUPAC Name(2Z)-4,6-dioxooct-2-enedioic acid
Traditional Namemaleylacetoacetic acid
SMILESOC(=O)CC(=O)CC(=O)\C=C\C(O)=O
InChI IdentifierInChI=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+
InChI KeyGACSIVHAIFQKTC-OWOJBTEDSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as medium-chain keto acids and derivatives. These are keto acids with a 6 to 12 carbon atoms long side chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassMedium-chain keto acids and derivatives
Direct ParentMedium-chain keto acids and derivatives
Alternative Parents
Substituents
  • Medium-chain keto acid
  • Beta-keto acid
  • 1,3-diketone
  • Beta-hydroxy ketone
  • Dicarboxylic acid or derivatives
  • Unsaturated fatty acid
  • 1,3-dicarbonyl compound
  • Fatty acyl
  • Enone
  • Acryloyl-group
  • Alpha,beta-unsaturated ketone
  • Ketone
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organooxygen compound
  • Organic oxide
  • Organic oxygen compound
  • Carbonyl group
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Biological Properties
StatusDetected and Not Quantified
OriginEndogenous
Cellular Locations
  • Cytoplasm
  • Extracellular
Biofluid LocationsNot Available
Tissue LocationsNot Available
Pathways
NameSMPDB LinkKEGG Link
Phenylalanine and Tyrosine MetabolismSMP00008 map00360
Tyrosine MetabolismSMP00006 map00350
Tyrosinemia Type ISMP00218 Not Available
ApplicationsNot Available
Biological RolesNot Available
Chemical RolesNot Available
Physical Properties
StateSolid
AppearanceWhite powder.
Experimental Properties
PropertyValue
Melting PointNot Available
Boiling PointNot Available
SolubilityNot Available
Predicted Properties
PropertyValueSource
Water Solubility1.65 g/LALOGPS
logP-0.19ALOGPS
logP0.4ChemAxon
logS-2.1ALOGPS
pKa (Strongest Acidic)2.72ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area108.74 ŲChemAxon
Rotatable Bond Count6ChemAxon
Refractivity44.4 m³·mol⁻¹ChemAxon
Polarizability16.75 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectra
Toxicity Profile
Route of ExposureNot Available
Mechanism of ToxicityNot Available
MetabolismNot Available
Toxicity ValuesNot Available
Lethal DoseNot Available
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Uses/SourcesThis is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.
Minimum Risk LevelNot Available
Health EffectsNot Available
SymptomsNot Available
TreatmentNot Available
Concentrations
Not Available
External Links
DrugBank IDNot Available
HMDB IDHMDB0002052
FooDB IDFDB022817
Phenol Explorer IDNot Available
KNApSAcK IDC00007508
BiGG ID1485279
BioCyc ID4-MALEYL-ACETOACETATE
METLIN ID6462
PDB IDNot Available
Wikipedia Link4-Maleylacetoacetic_acid
Chemspider ID4444078
ChEBI ID47904
PubChem Compound ID5280393
Kegg Compound IDC01036
YMDB IDNot Available
ECMDB IDM2MDB004846
References
Synthesis ReferenceNot Available
MSDSNot Available
General References
1. Kvittingen EA, Halvorsen S, Jellum E: Deficient fumarylacetoacetate fumarylhydrolase activity in lymphocytes and fibroblasts from patients with hereditary tyrosinemia. Pediatr Res. 1983 Jul;17(7):541-4.
2. Gray RG, Patrick AD, Preston FE, Whitfield MF: Acute hereditary tyrosinaemia type I: clinical, biochemical and haematological studies in twins. J Inherit Metab Dis. 1981;4(1):37-40.