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Record Information
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-27 20:29:59 UTC
Secondary Accession Numbers
  • HMDB00635
Metabolite Identification
Common NameSuccinylacetone
DescriptionSuccinylacetone, also known as 4,6-dioxoheptanoic acid or SUAC, belongs to the class of compounds known as medium-chain keto acids and derivatives. These are keto acids with 6 to 12 carbon atoms. Succinylacetone is soluble (in water) and a weakly acidic compound (based on its pKa). Succinylacetone has been detected in amniotic fluid, blood, and urine. Within the cell, succinylacetone is primarily located in the cytoplasm (predicted from logP). Succinylacetone can be created by the oxidation of glycine, and is a precursor of methylglyoxal (Wikipedia ). Succinylacetone is an abnormal tyrosine metabolite that arises from defects in the enzyme called fumarylacetoacetase (PMID: 16448836 ). Fumarylacetoacetase normally catalyzes the hydrolysis of 4-fumarylacetoacetate into fumarate and acetoacetate. If present in sufficiently high levels, succinylacetone 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 succinylacetone are associated with 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. Patients usually develop features such as hepatic necrosis, renal tubular injury, and hypertrophic cardiomyopathy. Neurologic and dermatologic manifestations are also possible. The urine has an odour of cabbage or rancid butter. Succinylacetone is a keto-acid, which is a subclass of organic acids. 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. Succinylacetone 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 ).
4,6-Dioxoheptanoic acidHMDB
Chemical FormulaC7H10O4
Average Molecular Weight158.1519
Monoisotopic Molecular Weight158.057908808
IUPAC Name4,6-dioxoheptanoic acid
Traditional Namesuccinylacetone
CAS Registry Number51568-18-4
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
  • 1,3-diketone
  • 1,3-dicarbonyl compound
  • Ketone
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Physiological effect

Health effect:



Biological location:


Indirect biological role:

Biological role:

Physical Properties
Experimental Properties
Melting Point66 - 67 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility24.1 g/LALOGPS
pKa (Strongest Acidic)4.15ChemAxon
pKa (Strongest Basic)-7.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area71.44 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity36.98 m³·mol⁻¹ChemAxon
Polarizability15.07 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-000f-9200000000-418fe15f4a3ff86cd732Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dl-9510000000-9b8725af310299084be0Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0a4i-0900000000-bc1ed64644f1d5262a67Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0a4l-4900000000-4a799061d93a46eb98abSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0a4i-1900000000-bb8ea63020dfb2d73fd6Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a4i-1900000000-cfd9bc7542d69961cfdeSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0a4r-5900000000-3abd8a41f7a1bab5e9a2Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9200000000-63209abdb7044cb484b2Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-006x-1900000000-78eabe7ae2f320bd578fSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0kml-7900000000-db14b02751e17fac3323Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9100000000-b6e51a674fbdfbbe701aSpectrum
MSMass Spectrum (Electron Ionization)splash10-000f-9100000000-a3b92f310030718acb91Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Amniotic Fluid
  • Blood
  • Urine
Tissue LocationsNot 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 Quantified0-10 umol/mmol creatinineNewborn (0-30 days old)Both
UrineDetected and Quantified2.8 (0.6-4.7) umol/mmol creatinineAdult (>18 years old)Both
UrineDetected and Quantified8.329 +/- 5.467 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    • Mordechai, Hien, ...
Abnormal Concentrations
BloodDetected and Quantified327.300 +/- 125.8(190-543) uMChildren (1-13 years old)BothTyrosinemia I details
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 Quantified9.683 +/- 7.284 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
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. Han LS, Ye J, Qiu WJ, Zhang HW, Wang Y, Ji WJ, Gao XL, Li XY, Jin J, Gu XF: [Application of succinylacetone levels measurement in the blood and urine in the diagnosis of tyrosinemia type 1]. Zhonghua Er Ke Za Zhi. 2012 Feb;50(2):126-30. [PubMed:22455637 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: METAGENE consortium.
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022156
KNApSAcK IDNot Available
Chemspider ID5121
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkSuccinylacetone
PubChem Compound5312
PDB IDNot Available
ChEBI ID87897
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB ID
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. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  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 ]
  8. 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 ]