Succinylacetone

Succinylacetone
Names
Preferred IUPAC name
4,6-Dioxoheptanoic acid
Other names
Succinyl acetone
Identifiers
CAS Number
  • 51568-18-4 checkY
3D model (JSmol)
  • Interactive image
ECHA InfoCard 100.153.292 Edit this at Wikidata
PubChem CID
  • 5312
UNII
  • KZ0KV2Q190 checkY
CompTox Dashboard (EPA)
  • DTXSID50199519 Edit this at Wikidata
InChI
  • InChI=1S/C7H10O4/c1-5(8)4-6(9)2-3-7(10)11/h2-4H2,1H3,(H,10,11)
    Key: WYEPBHZLDUPIOD-UHFFFAOYSA-N
  • CC(=O)CC(=O)CCC(=O)O
Properties
Chemical formula
 C7H10O4
Molar mass 158.153 g·mol−1
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
Infobox references
Chemical compound

Succinylacetone is a chemical compound that is formed by the oxidation of glycine and is a precursor of methylglyoxal. It is a pathognomonic compound found in the urine of patients with tyrosinemia type 1,[1] which is due to congenital deficiency of an enzyme, fumarylacetoacetate hydrolase. This enzyme is involved in the catabolism of tyrosine, and if deficient, leads to accumulation of fumarylacetoacetate which is subsequently converted to succinylacetone which can be detected in the urine by GCMS. Succinylacetone also inhibits ALA dehydratase (PBG synthase) which increases ALA and precipitates acute neuropathic symptoms, similar to porphyria.

References

  1. ^ de Laet C, Dionisi-Vici C, Leonard JV, McKiernan P, Mitchell G, Monti L, et al. (January 2013). "Recommendations for the management of tyrosinaemia type 1". Orphanet Journal of Rare Diseases. 8: 8. doi:10.1186/1750-1172-8-8. PMC 3558375. PMID 23311542.