3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria |
| |
| Authors: | W. Vogt P. Sandel Ch. Langfelder M. Knedel |
| |
| Affiliation: | Institut für Klinische Chemie am Klinikum Grosshadern der Universität München, MünchenG.F.R. |
| |
| Abstract: | 1. 3-Methylcrotonylglycine was identified in urine from an infant with 3-hydroxy-3-methylglutaric aciduria. 2. The concentration of 3-methylcrotonylglycine in urine was approximately one sixth of that of the other metabolite of 3-methylcrotonyl-CoA, 3-hydroxyisovaleric acid. 3. The presence of both metabolites in the infant's urine indicates an inhibition of 3-methylcrotonyl-CoA carboxylase activity in tissues of the infant. |
| |
| Keywords: | To whom correspondence should be addressed. |
| 本文献已被 ScienceDirect PubMed 等数据库收录！ |
