- Metabolite
- HMDB0003771 (2-Methylacetoacetic acid)
- Biospecimen
- Urine
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- 3368 umol/mmol creatinine
- Age
- Children (1-13 years old)
- Sex
- Not Specified
- Condition
- Beta-ketothiolase deficiency
Comments
with L-carnitine-supplement
References
- Fontaine M, Briand G, Ser N, Armelin I, Rolland MO, Degand P, Vamecq J: Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency. Clin Chim Acta. 1996 Nov 15;255(1):67-83. [PubMed:8930414 ]