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Record Information
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-05-29 19:28:08 UTC
Secondary Accession NumbersNone
Metabolite Identification
Common NameSuccinylacetone
DescriptionSuccinylacetone is a tyrosine metabolite (PMID 16448836 ). It is a specific marker for Tyrosinemia type I. Type I tyrosinemia is an inherited metabolism disorder due to a shortage of the enzyme fumarylacetoacetate hydrolase that is needed to break down tyrosine. (Wikipedia).
  1. 4,6-Dioxoheptanoate
  2. 4,6-Dioxoheptanoic acid
  3. Succinylacetone
Chemical FormulaC7H10O4
Average Molecular Weight158.1519
Monoisotopic Molecular Weight158.057908808
IUPAC Name4,6-dioxoheptanoic acid
Traditional Name4,6-dioxoheptanoic acid
CAS Registry Number51568-18-4
InChI Identifier
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassOrganic Acids and Derivatives
ClassKeto-Acids and Derivatives
Sub ClassMedium-chain Keto Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Organic Compounds
  • Straight Chain Fatty Acids
  • Carboxylic Acid
  • Gamma Keto Acid
  • Ketone
Direct ParentMedium-chain Keto Acids and Derivatives
StatusDetected and Quantified
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
Experimental Properties
Melting Point66 - 67 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility24.1ALOGPS
pKa (Strongest Acidic)4.15ChemAxon
pKa (Strongest Basic)-7.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area71.44 Å2ChemAxon
Rotatable Bond Count5ChemAxon
Refractivity36.98 m3·mol-1ChemAxon
Polarizability15.07 Å3ChemAxon
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Amniotic Fluid
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
Amniotic FluidDetected and Quantified0.003 (0.00-0.013) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.15 (0.00-0.30) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.013 (0.003-0.021) uMAdult (>18 years old)BothNormal details
UrineDetected and Quantified2.8 (0.6-4.7) umol/mmol creatinineAdult (>18 years old)Both
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BloodDetected and Quantified3.3 (0.9-5.7) uMAdult (>18 years old)BothHepatorenal tyrosinemia type I details
BloodDetected and Quantified0.033 (0.021-0.055) uMAdult (>18 years old)Both
UrineDetected and Quantified360.00 (20.00-700.00) umol/mmol creatinineChildren (1-13 years old)BothTyrosinemia I details
Associated Disorders and Diseases
Disease References
  1. Cyr D, Giguere R, Villain G, Lemieux B, Drouin R: A GC/MS validated method for the nanomolar range determination of succinylacetone in amniotic fluid and plasma: an analytical tool for tyrosinemia type I. J Chromatogr B Analyt Technol Biomed Life Sci. 2006 Feb 17;832(1):24-9. Epub 2006 Jan 18. Pubmed: 16414314
Tyrosinemia I
  1. MetaGene
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022156
KNApSAcK IDNot Available
Chemspider ID5121
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00635
Metagene LinkHMDB00635
PubChem Compound5312
ChEBI IDNot Available
Synthesis Reference Levenson, Corey H. Process for the preparation of succinylacetone. PCT Int. Appl. (1991), 9 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Fernandez-Canon JM, Baetscher MW, Finegold M, Burlingame T, Gibson KM, Grompe M: Maleylacetoacetate isomerase (MAAI/GSTZ)-deficient mice reveal a glutathione-dependent nonenzymatic bypass in tyrosine catabolism. Mol Cell Biol. 2002 Jul;22(13):4943-51. Pubmed: 12052898
  2. Laberge C, Lescault A, Grenier A, Morrisette J, Gagne R, Gadbois P, Halket J: Oral loading of homogentisic acid in controls and in obligate heterozygotes for hereditary tyrosinemia type I. Am J Hum Genet. 1990 Aug;47(2):329-37. Pubmed: 2378359
  3. Poudrier J, Lettre F, St-Louis M, Tanguay RM: Genotyping of a case of tyrosinaemia type I with normal level of succinylacetone in amniotic fluid. Prenat Diagn. 1999 Jan;19(1):61-3. Pubmed: 10073910
  4. Jakobs C, Dorland L, Wikkerink B, Kok RM, de Jong AP, Wadman SK: Stable isotope dilution analysis of succinylacetone using electron capture negative ion mass fragmentography: an accurate approach to the pre- and neonatal diagnosis of hereditary tyrosinemia type I. Clin Chim Acta. 1988 Feb 15;171(2-3):223-31. Pubmed: 3286060
  5. Kimura A, Endo F, Kagimoto S, Inoue T, Suzuki M, Kurosawa T, Tohma M, Fujisawa T, Kato H: Tyrosinemia type I-like disease: a possible manifestation of 3-oxo-delta 4-steroid 5 beta-reductase deficiency. Acta Paediatr Jpn. 1998 Jun;40(3):211-7. Pubmed: 9695292
  6. Magera MJ, Gunawardena ND, Hahn SH, Tortorelli S, Mitchell GA, Goodman SI, Rinaldo P, Matern D: Quantitative determination of succinylacetone in dried blood spots for newborn screening of tyrosinemia type I. Mol Genet Metab. 2006 May;88(1):16-21. Epub 2006 Jan 31. Pubmed: 16448836
  7. Endo F, Katoh H, Yamamoto S, Matsuda I: A murine model for type III tyrosinemia: lack of immunologically detectable 4-hydroxyphenylpyruvic acid dioxygenase enzyme protein in a novel mouse strain with hypertyrosinemia. Am J Hum Genet. 1991 Apr;48(4):704-9. Pubmed: 2014797