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Clinical data | |
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AHFS/Drugs.com | Consumer Drug Information |
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Pharmacokinetic data | |
Metabolism | to phenylacetic acid |
Elimination half-life | 0.8 hours |
Excretion | 80% as phenylacetylglutamine |
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ECHA InfoCard | 100.130.318 |
Chemical and physical data | |
Formula | C10H11NaO2 |
Molar mass | 186.2 g/mol g·mol−1 |
3D model (JSmol) | |
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Sodium phenylbutyrate is an orphan drug, marketed by Ucyclyd Pharma (Hunt Valley, USA) under the trade name Buphenyl, by Swedish Orphan International (Sweden) as Ammonaps and by Fyrlklövern Scandinavia as triButyrate.
Phenylbutyrate has been used to treat urea cycle disorders.[1]
Metabolism
Phenylbutyrate is a prodrug. In the human body it is metabolized by beta-oxidation to phenylacetate.
Phenylacetate conjugates with glutamine to phenylacetylglutamine, that is eliminated with the urine.
![](https://upload.wikimedia.org/wikipedia/commons/thumb/f/fd/Nitrogen_elimination.jpg/300px-Nitrogen_elimination.jpg)
Research
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.[citation needed]
Phenylbutyrate has been associated with longer lifespans in Drosophila.[2]
University of Colorado researchers Dr. Curt Freed and Wenbo Zhou demonstrated that phenylbutyrate stops the progression of Parkinson's disease in mice by turning on a gene called DJ-1 that can protect dopaminergic neurons in the midbrain from dying. As of July 2011 they plan on testing phenylbutyrate for the treatment of Parkinson's disease in humans.[3]
References
- ^ Batshaw, M. L.; MacArthur, R. B.; Tuchman, M. (2001). "Alternative pathway therapy for urea cycle disorders: twenty years later". J. Pediatr. 138 (1 Suppl): S46–S54, discussion S54–S55. doi:10.1067/mpd.2001.111836. PMID 11148549.
- ^ Kang, H. L.; Benzer, S.; Min, K. T. (2002). "Life extension in Drosophila by feeding a drug". Proc. Natl. Acad. Sci. U.S.A. 99 (2): 838–843. doi:10.1073/pnas.022631999. PMC 117392. PMID 11792861.
- ^ News Article