Sodium phenylbutyrate
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Sodium phenylbutyrate
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| Systematic (IUPAC) name | |
| Sodium 4-phenylbutanoate | |
| Identifiers | |
| CAS number | |
| ATC code | A16 |
| PubChem | |
| Chemical data | |
| Formula | C10H11NaO2 |
| Mol. mass | 186.2 g/mol |
| Pharmacokinetic data | |
| Bioavailability | ? |
| Metabolism | to phenylacetate |
| Half life | 0.8 hours |
| Excretion | 80% as phenylacetylglutamine |
| Therapeutic considerations | |
| Licence data |
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| Pregnancy cat. |
not to be used |
| Legal status | |
| Routes | ? |
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®. 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.
Sodium Phenylbutyrate Powder is supplied worldwide by Scandinavian Formulas, Inc. (Sellersville, PA 18960 USA)to clinics, hospitals and universities.
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.
[edit] References
1. Batshaw ML, et al. Alternative pathway therapy for urea cycle disorders: twenty years later. J Pediatr 2001; 138 (suppl 1): S46-S55. (PubMed id:11148549) Correction. ibid. 2002; 140: 490.
2. Kang HL, Benzer S, Min KT.; Life extension in Drosophila by feeding a drug.; Proc Natl Acad Sci U S A. 2002 Jan 22; 99(2):838-43. Epub 2002 Jan 15.; PMID: 11792861
