Succinylacetone
Jump to navigation
Jump to search
![]() | |
Names | |
---|---|
Preferred IUPAC name
4,6-Dioxoheptanoic acid | |
Other names
Succinyl acetone
| |
Identifiers | |
3D model (JSmol)
|
|
PubChem CID
|
|
UNII | |
| |
| |
Properties | |
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).
|
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, 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
Categories:
- Articles lacking sources from November 2020
- All articles lacking sources
- Chemical pages without ChemSpiderID
- Articles without EBI source
- Articles without KEGG source
- Pages using collapsible list with both background and text-align in titlestyle
- Articles containing unverified chemical infoboxes
- Chembox image size set
- Articles with short description
- Short description with empty Wikidata description
- Diketones
- Carboxylic acids