07/01/1979 - "Data are presented to show that the unusual ketone, 3-pentanone, which has been observed previously in the urine of patients with propionic acidemia, is produced as a result of the decarboxylation of 3-keto-2-methylvaleric acid."
07/01/1979 - "Two abnormal metabolites, 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid, have been identified and quantitated in the urine of a child with propionic acidemia. "
07/01/1979 - "The identification of 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid in a patient with propionic acidemia."
04/01/1981 - "Propionyl-CoA carboxylase deficiency: case report, effect of low-protein diet and identification of 3-oxo-2-methylvaleric acid 3-hydroxy-2-methylvaleric acid, and maleic acid in urine."
|2.||maleic acid (maleate)
|1.||Protein-Restricted Diet (Diet, Protein Restricted)