产品描述:
Glycerol phenylbutyrate is a pre-prodrug form of the nitrogen-binding prodrug and chemical chaperone sodium 4-phenylbutyrate (Item No. 11323).{69779} It is converted by pancreatic lipase in the small intestine to sodium 4-phenylbutyrate, which is subsequently converted into the active metabolite phenylacetate via β-oxidation. Formulations containing glycerol phenylbutyrate have been used in the treatment of urea cycle disorders that cannot be managed by diet or amino acid supplementation alone.