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Clinical data | |
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AHFS/Drugs.com | Consumer Drug Information |
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ATC code | |
Pharmacokinetic data | |
Metabolism | to phenylacetic acid |
Elimination half-life | 0.8 hours |
Excretion | 80% as phenylacetylglutamine |
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CompTox Dashboard (EPA) | |
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 and by Swedish Orphan International (Sweden) as Ammonaps.
It has been used to treat urea cycle disorders.[1]
On July 5, 2011, Colorado researchers, Dr. Curt Freed and Wenbo Zhou, have discovered a drug that stops the progression of Parkinson's disease in mice and is now being tested on humans. They have found that the drug phenylbutyrate turns on a gene, called DJ-1, that can protect dopamine neurons in Parkinson's disease. Parkinson's disease is caused by dying midbrain dopamine neurons.[2]
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.
PBA has been associated with longer lifespans in Drosophila.[3]
References
- ^ Batshaw ML, MacArthur RB, Tuchman M (2001). "Alternative pathway therapy for urea cycle disorders: twenty years later". J. Pediatr. 138 (1 Suppl): S46–54, discussion S54–5. doi:10.1067/mpd.2001.111836. PMID 11148549.
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: CS1 maint: multiple names: authors list (link) - ^ News Article
- ^ Kang HL, Benzer S, Min KT (2002). "Life extension in Drosophila by feeding a drug". Proc. Natl. Acad. Sci. U.S.A. 99 (2): 838–43. doi:10.1073/pnas.022631999. PMC 117392. PMID 11792861.
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: CS1 maint: multiple names: authors list (link)