Succinylacetone

Succinylacetone
Names
	Preferred IUPAC name 4,6-Dioxoheptanoic acid
	Other names Succinyl acetone
Identifiers
CAS Number	51568-18-4 ;
3D model (JSmol)	Interactive image;
ECHA InfoCard	100.153.292
PubChem CID	5312;
UNII	KZ0KV2Q190 ;
CompTox Dashboard (EPA)	DTXSID50199519 ;
	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;
	SMILES 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

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

^ 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.

[de_Laet_2013-1] 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.

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