3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria.
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.
D003437:Crotonates / Q000652:urine; D005977:Glutarates / Q000652:urine*; D005998:Glycine / Q000031:analogs & derivatives* / Q000138:chemical synthesis / Q000652:urine; D006801:Humans; D006880:Hydroxy Acids / Q000652:urine; D007223:Infant; D008297:Male; D008661:Metabolism, Inborn Errors / Q000652:urine*
D003437:Crotonates; D005977:Glutarates; D006880:Hydroxy Acids; C002163:beta-methylcrotonylglycine; D005998:Glycine