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Sodium phenylbutyrate
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®.
It has been used to treat urea cycle disorders.[1]
Scanadinavian Formulas, Inc. Sellersville, PA supplies Sodium Phenylbutyrate worldwide for clinical trials.
Metabolism
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.
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.[2]
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. PMID 11148549.
- ^ 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. PMID 11792861.
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Sodium_phenylbutyrate". A list of authors is available in Wikipedia.
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