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Record Information
StatusExpected but not Quantified
Creation Date2018-02-28 20:41:01 UTC
Update Date2019-07-23 14:38:58 UTC
Secondary Accession NumbersNone
Metabolite Identification
Common NameSuccinylacetoacetate
DescriptionSuccinylacetoacetate is an intermediate that arises due to the incomplete breakdown of tyrosine (PMID: 7171740 ). It is produced as a result of defects in the enzyme called fumarylacetoacetase. Fumarylacetoacetase normally catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. If present in sufficiently high levels, succinylacetoacetate can act as an acidogen, an oncometabolite, and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. An oncometabolite is an endogenous metabolite that causes cancer. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of succinylacetoacetate are associated with tyrosinemia type I. Type I tyrosinemia is an inherited metabolic disorder due to a shortage of the enzyme fumarylacetoacetate hydrolase that is needed to break down tyrosine. Patients usually develop features such as hepatic necrosis, renal tubular injury, and hypertrophic cardiomyopathy. Neurologic and dermatologic manifestations are also possible as well as hepatocellular carcinoma. The urine of tyrosinemia patients has an odour of cabbage or rancid butter. Succinylacetoacetate is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated tyrosinemia. Many affected children with organic acidemias experience intellectual disability or delayed development. Succinylacetoacetate appears to function as an oncometabolite (similar in function to succinate, another oncometabolite) as patients with high levels of this compound often develop hepatocellular carcinoma (PMID: 20003495 ).
3,5-Dioxosuberic acidChEBI
Succinyl-acetoacetic acidGenerator
Succinylacetoacetic acidGenerator
Succinyl acetoacetic acidHMDB
3,5-Dioxooctanedioic acidHMDB
Chemical FormulaC8H10O6
Average Molecular Weight202.1614
Monoisotopic Molecular Weight202.047738052
IUPAC Name3,5-dioxooctanedioic acid
Traditional Name3,5-dioxooctanedioic acid
CAS Registry Number65115-74-4
InChI Identifier
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
  • Medium-chain keto acid
  • Gamma-keto acid
  • Beta-keto acid
  • 1,3-diketone
  • Beta-hydroxy ketone
  • Dicarboxylic acid or derivatives
  • 1,3-dicarbonyl compound
  • Ketone
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organooxygen compound
  • Hydrocarbon derivative
  • Carbonyl group
  • Organic oxide
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available

Indirect biological role:

Biological role:

Physical Properties
StateNot Available
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
pKa (Strongest Acidic)3.46ChemAxon
pKa (Strongest Basic)-7.3ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area108.74 ŲChemAxon
Rotatable Bond Count7ChemAxon
Refractivity43.33 m³·mol⁻¹ChemAxon
Polarizability17.72 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0f79-1920000000-22c7089e732e52f51956Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-5900000000-3ca1ab6d1f60b5276b83Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-05mo-9200000000-27c4746155a2bf563b4dSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0zfr-2950000000-b298b52855b7e52a39d3Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0a4i-5900000000-7f946ff237e2d2e0574dSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9500000000-8273967ad8e55cae9074Spectrum
Biological Properties
Cellular LocationsNot Available
Biospecimen LocationsNot Available
Tissue LocationsNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDNot Available
KNApSAcK IDNot Available
Chemspider IDNot Available
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound125181
PDB IDNot Available
ChEBI ID87999
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB ID
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Lindblad B, Steen G: Identification of 4,6-dioxoheptanoic acid (succinylacetone), 3,5-dioxooctanedioic acid (succinylacetoacetate) and 4-Oxo-6-hydroxyheptanoic acid in the urine from patients with hereditary tyrosinemia. Biomed Mass Spectrom. 1982 Oct;9(10):419-24. [PubMed:7171740 ]
  2. Cassiman D, Zeevaert R, Holme E, Kvittingen EA, Jaeken J: A novel mutation causing mild, atypical fumarylacetoacetase deficiency (Tyrosinemia type I): a case report. Orphanet J Rare Dis. 2009 Dec 15;4:28. doi: 10.1186/1750-1172-4-28. [PubMed:20003495 ]