Metabolite Card
Formula: C5H8O3 (116.0473)
SMILES: C[C@H](C(C)=O)C(O)=O
Synonyms [en]
2methyl-3-ketovaleric acid; 2-methyl-3-oxo-butyric acid; alpha-Methylacetoacetic acid; 2-Methyl-3-oxobutanoic acid; 2-Methyl-3-oxo-butanoic acid; 2-Methylacetoacetic acid
Last reviewed on 2024-06-28.
Cite this Page
2-Methylacetoacetic acid. 数据之源，洞见之始. SMRUCC genomics institute, a synthetic life researcher from China.
https://biocad_registry.innovation.ac.cn/s/(-)-arctiin
(retrieved
2026-01-03) (CAD Registry RN: BioCAD00000020305). Licensed
under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).
Note
2-Methylacetoacetic acid (CAS: 2382-59-4) is a metabolite that has an increased excretion in patients with acetoacetyl-CoA thiolase (EC 2.3.1.9) deficiency (OMIM: 607809, ACAT1; OMIM: 100678, ACAT2; OMIM: 604770, ACAA2; OMIM: 607809). Thiolases are ubiquitous and important enzymes. Several isoenzymes are known, which can occur in the cytosol (ACAT2), the mitochondria (ACAT1), or the peroxisomes (ACAA2). Thiolases are CoA-dependent enzymes which catalyze the formation of a carbon-carbon bond in a Claisen condensation step and its reverse reaction via a thiolytic degradation mechanism. Mitochondrial acetoacetyl-coenzyme A (CoA) thiolase (T2) is important in the pathways for the synthesis and degradation of ketone bodies as well as for the degradation of 2-methylacetoacetyl-CoA (PMID: 17371050, 14518824, 9744475, 9001814). Moreover, 2-methylacetoacetic acid is found to be associated with beta-ketothiolase deficiency, which is also an inborn error of metabolism. 2-Methylacetoacetic acid is found in urine and can be used as a biomarker for the diagnosis of beta-ketothiolase deficiency.
DBLinks
- CAS Registry Number: 76652-88-5
- PubChem CID: 150996
- ChEBI: 37079
- HMDB: HMDB0003771
- LipidMaps: LMFA01060192
- KEGG:
- BioCyc:
- NCBI MeSH: 2-methylacetoacetic acid
- Wikipedia:
Other DBLinks
- CAS Registry Number: 2382-59-4
- CAS Registry Number: 76652-86-3
- CAS Registry Number: 76652-88-5
- PubChem: 150996
- PubChem: 40578931
- ChEBI: ChEBI:37079
- HMDB: HMDB0003771
- LipidMaps: LMFA01060192
- NCBI MeSH: 2-methylacetoacetic acid
- Metlin: METLIN_45873
- Coconut NaturalProduct: CNP0002342.0
Class / Ontology
- WishartLab ClassyFire: [Short-chain keto acids and derivatives] Short-chain keto acids and derivatives
- LipidMaps: [Oxo fatty acids [FA0106]] Oxo fatty acids [FA0106]
- Coconut NaturalProduct: [Oxo fatty acids] Oxo fatty acids
Taxonomy Source
Pathway Synthetic
| pathway id | name |
|---|---|
| WikiPathways:WP4686 | Leucine, isoleucine and valine metabolism |