- Metabolite
- HMDB0000019 (alpha-Ketoisovaleric acid)
- Biospecimen
- Urine
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- 550.00 (300.00-800.00) umol/mmol creatinine
- Age
- Children (1-13 years old)
- Sex
- Both
- Condition
- Maple syrup urine disease (MSUD). When treated, the concentrations are 1-2 uM/mM.
References
- G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.