- Metabolite
- HMDB0000407 (2-Hydroxy-3-methylbutyric acid)
- Biospecimen
- Cerebrospinal Fluid (CSF)
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- 809 uM
- Age
- Newborn (0-30 days old)
- Sex
- Female
- Condition
- Maple syrup urine disease
References
- Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ]