File:Phbutyrate.jpg | |
Clinical data | |
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ATC code | |
Pharmacokinetic data | |
Metabolism | to phenylacetate |
Excretion | 80% as phenylacetylglutamine |
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CAS Number | |
CompTox Dashboard (EPA) | |
ECHA InfoCard | 100.130.318 |
Chemical and physical data | |
Formula | C10H11NaO2 |
Molar mass | 186.2 g·mol−1 |
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 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.
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