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Glycerol phenylbutyrate is a pre-prodrug form of the nitrogen-binding prodrug and chemical chaperone sodium 4-phenylbutyrate (Item No. 11323).1 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.
WARNING This product is not for human or veterinary use.
1. Glycerol phenylbutyrate for the chronic management of urea cycle disorders. Expert Rev. Endocrinol. Metab. 9(5), 427-434 (2014).