Glycerol phenylbutyrate

• CAT Number: I006985
• CAS Number: 611168-24-2
• Molecular Formula: C33H38O6
• Molecular Weight: 530.661
• Purity: ≥95%
• Synonyms: HPN-100; HPN 100; HPN100; Glycerol phenylbutyrate, trade name: Ravicti.;propane-1,2,3-triyl tris(4-phenylbutanoate)
• Target: Sigma Receptor
• Solubility: Soluble in DMSO, not in water
• Storage: 0 - 4°C for short term or -20 °C for long term
• IUPAC Name: 2,3-bis(4-phenylbutanoyloxy)propyl 4-phenylbutanoate
• InChI: InChI=1S/C33H38O6/c34-31(22-10-19-27-13-4-1-5-14-27)37-25-30(39-33(36)24-12-21-29-17-8-3-9-18-29)26-38-32(35)23-11-20-28-15-6-2-7-16-28/h1-9,13-18,30H,10-12,19-26H2
• InChIKey: ZSDBFLMJVAGKOU-UHFFFAOYSA-N
• SMILES: C1=CC=C(C=C1)CCCC(=O)OCC(COC(=O)CCCC2=CC=CC=C2)OC(=O)CCCC3=CC=CC=C3

Glycerol phenylbutyrate is a prodrug used in the management of urea cycle disorders (UCDs), conditions in which the body cannot effectively remove ammonia, a toxic byproduct of protein metabolism. Once administered, glycerol phenylbutyrate is metabolized into phenylbutyrate, which is then converted to phenylacetate in the liver. Phenylacetate conjugates with glutamine to form phenylacetylglutamine, which is excreted in the urine, thereby removing excess nitrogen from the body. This helps to lower ammonia levels in patients with UCDs, preventing hyperammonemia and associated neurological damage.