Human Metabolome Database Version 3.5

Showing metabocard for Succinylacetone (HMDB00635)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:28:08 -0600
HMDB ID HMDB00635
Secondary Accession Numbers None
Metabolite Identification
Common Name Succinylacetone
Description Succinylacetone is a tyrosine metabolite (PMID 16448836 Link_out). 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).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 4,6-Dioxoheptanoate
  2. 4,6-Dioxoheptanoic acid
  3. Succinylacetone
Chemical Formula C7H10O4
Average Molecular Weight 158.1519
Monoisotopic Molecular Weight 158.057908808
IUPAC Name 4,6-dioxoheptanoic acid
Traditional IUPAC Name 4,6-dioxoheptanoic acid
CAS Registry Number 51568-18-4
SMILES CC(=O)CC(=O)CCC(O)=O
InChI Identifier InChI=1S/C7H10O4/c1-5(8)4-6(9)2-3-7(10)11/h2-4H2,1H3,(H,10,11)
InChI Key WYEPBHZLDUPIOD-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Organic Acids and Derivatives
Class Keto-Acids and Derivatives
Sub Class Medium-chain Keto Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Organic Compounds
  • Straight Chain Fatty Acids
Substituents
  • Carboxylic Acid
  • Gamma Keto Acid
  • Ketone
Direct Parent Medium-chain Keto Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction Not Available
Application Not Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 66 - 67 °C Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 24.1 g/L ALOGPS
LogP -0.18 ALOGPS
LogP 0.15 ChemAxon
LogS -0.82 ALOGPS
pKa (strongest acidic) 4.15 ChemAxon
pKa (strongest basic) -7.2 ChemAxon
Hydrogen Acceptor Count 4 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 71.44 A2 ChemAxon
Rotatable Bond Count 5 ChemAxon
Refractivity 36.98 ChemAxon
Polarizability 15.07 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Amniotic Fluid
  • Blood
  • Urine
Tissue Location Not Available
Pathways Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Amniotic Fluid Detected and Quantified
0.003 (0.00-0.013) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
0.15 (0.00-0.30) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
0.013 (0.003-0.021) uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
2.8 (0.6-4.7) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
1.0 (0.0-2.0) umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 3.3 (0.9-5.7) uM Adult (>18 years old) Both Hepatorenal tyrosinemia type I
Blood Detected and Quantified 0.033 (0.021-0.055) uM Adult (>18 years old) Both Comment Tyrosinemia
Urine Detected and Quantified 360.00 (20.00-700.00) umol/mmol creatinine Children (1-13 year old) Both Tyrosinemia I
Associated Disorders and Diseases
Disease References
Tyrosinemia
  • 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 Link_out
      Tyrosinemia I
          Associated OMIM IDs
          DrugBank ID Not Available
          DrugBank Metabolite ID Not Available
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB022156
          KNApSAcK ID Not Available
          Chemspider ID 5121 Link_out
          KEGG Compound ID Not Available
          BioCyc ID Not Available
          BiGG ID Not Available
          Wikipedia Link Not Available
          NuGOwiki Link HMDB00635 Link_out
          Metagene Link HMDB00635 Link_out
          METLIN ID 5608 Link_out
          PubChem Compound 5312 Link_out
          PDB ID SHU Link_out
          ChEBI ID Not Available
          References
          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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out
          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 Link_out