Sodium phenylbutyrate

Sodium phenylbutyrate is an orphan drug, marketed by Ucyclyd Pharma (Hunt Valley, USA) under the trade name Buphenyl® and by Orphan Europe (France) as Ammonaps®.

It has been used to treat urea cycle disorders.

Metabolism
Phenylbutyrate is a prodrug. In the human body it is metabolized by beta-oxidation to phenylacetate.

Phenylacetate conjugate with glutamine to phenylacetylglutamine, that is eliminated with the urine.



Research
Sodium phenylbutyrate is also under investigation for the treatment of some sickle-cell disorders (Blood Products Plasma Expanders and Haemostatics) and for use as a potential differentiation-inducing agent in malignant glioma and acute myeloid leukaemia.

PBA has been associated with longer lifespans in Drosophila.