- Metabolite
- HMDB0000754 (3-Hydroxyisovaleric acid)
- Biospecimen
- Urine
- Status
- Detected and Quantified
- Data source
- Referenced
- Concentration
- 2974 umol/mmol creatinine
- Age
- Children (1 - 13 years old)
- Sex
- Not Specified
- Condition
- Isolated 3-Methylcrotonyl-CoA Carboxylase Deficiency
References
- de Kremer RD, Latini A, Suormala T, Baumgartner ER, Larovere L, Civallero G, Guelbert N, Paschini-Capra A, Depetris-Boldini C, Mayor CQ: Leukodystrophy and CSF purine abnormalities associated with isolated 3-methylcrotonyl-CoA carboxylase deficiency. Metab Brain Dis. 2002 Mar;17(1):13-8. [PubMed:11893004 ]