2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.

Fluridone (EL-171) is a potent inhibitor of abscisic acid (ABA) synthesis, inhibits the expression of AchnFAR and TF genes, and reduces the formation of primary alcohol.

An imidazole derivative which is a metabolite of the antineoplastic agents BIC and DIC.