- Metabolite
- HMDB0000557 (L-Alloisoleucine)
- Biospecimen
- Blood
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- 25.6 +/- 14.0 uM
- Age
- Children (1 - 13 years old)
- Sex
- Not Specified
- Condition
- Maple syrup urine disease
Comments
Patients treated with a natural protein restricted diet with low BCAA and supplemented with a semi-synthetic formula of essential amino acids containing small amounts of vitamins and minerals
References
- Barschak AG, Marchesan C, Sitta A, Deon M, Giugliani R, Wajner M, Vargas CR: Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles. Clin Biochem. 2008 Mar;41(4-5):317-24. Epub 2007 Dec 5. [PubMed:18088602 ]