Related Experiment Videos
The identification of 3-keto-2-methylvaleric acid and 3-hydroxy-2-methylvaleric acid in a patient with propionic
Biomedical Mass Spectrometry
|July 1, 1979
Abstract:
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. These metabolites may be produced as a result of the self-condensation of propionyl-CoA. 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.