Glycerol Phenylbutyrate
Catalog No.
C8708
A triglyceride prodrug form for chronic hyperammonemia control in urea cycle disorders
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Glycerol phenylbutyrate (GPB, Ravicti) is a triglyceride prodrug form used for chronic hyperammonemia control in urea cycle disorders. After oral administration, it is hydrolyzed by lipases in the small intestine to release phenylbutyric acid (PBA), which is then converted via β‑oxidation to phenylacetic acid (PAA). PAA conjugates with glutamine in the liver and kidneys to form phenylacetylglutamine (PAGN), which contains two nitrogen atoms and is excreted in urine, effectively providing an “alternative nitrogen excretion pathway” that bypasses the urea cycle. Compared with earlier sodium salt formulations, GPB has better oral tolerability and more stable ammonia control.
| Storage | Store at -20°C |
| M.Wt | 530.66 |
| Cas No. | 611168-24-2 |
| Formula | C33H38O6 |
| Synonyms | HPN-100 |
| Chemical Name | propane-1,2,3-triyl tris(4-phenylbutanoate) |
| SDF | Download SDF |
| Canonical SMILES | O=C(OCC(OC(=O)CCCC=1C=CC=CC1)COC(=O)CCCC=2C=CC=CC2)CCCC=3C=CC=CC3 |
| Shipping Condition | Small Molecules with Blue Ice, Modified Nucleotides with Dry Ice. |
| General tips | We do not recommend long-term storage for the solution, please use it up soon. |