Description: Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239 ). Methylglutaric acid is also found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency,...

3-Methylglutaric acid is a metabolite of the essential amino acid L-leucine (Cay-34342). It is produced by hydrolysis of the L-leucine catabolic intermediate 3-methylglutaconyl-CoA (3-MG-CoA), which accumulates when the activity of 3-MG-CoA hydratase, the enzyme that converts 3-MG-CoA to HMG-CoA, is deficient, in...