Sodium phenylbutyrate, sold under the brand name Buphenyl among others, is a salt of an aromatic fatty acid, 4-phenylbutyrate (4-PBA) or 4-phenylbutyric acid.[7] The compound is used to treat urea cycle disorders, because its metabolites offer an alternative pathway to the urea cycle to allow excretion of excess nitrogen.[8][9]
Sodium phenylbutyrate is also a histone deacetylase inhibitor and chemical chaperone, leading respectively to research into its use as an anti-cancer agent and in protein misfolding diseases such as cystic fibrosis.[7]
^"Prescription medicines: registration of new chemical entities in Australia, 2017". Therapeutic Goods Administration (TGA). 21 June 2022. Retrieved 9 April 2023.
^"Prescription medicines and biologicals: TGA annual summary 2017". Therapeutic Goods Administration (TGA). 21 June 2022. Retrieved 31 March 2024.
^"Health Canada New Drug Authorizations: 2015 Highlights". Health Canada. 4 May 2016. Retrieved 7 April 2024.
^Cite error: The named reference Buphenyl FDA label was invoked but never defined (see the help page).
^"Olpruva - sodium phenylbutyrate kit". DailyMed. 22 December 2022. Retrieved 21 January 2023.
^"Ammonaps EPAR". European Medicines Agency. 17 September 2018. Retrieved 3 January 2023.
^ abIannitti T, Palmieri B (September 2011). "Clinical and experimental applications of sodium phenylbutyrate". Drugs in R&D. 11 (3): 227–249. doi:10.2165/11591280-000000000-00000. PMC 3586072. PMID 21902286.
^Batshaw ML, MacArthur RB, Tuchman M (January 2001). "Alternative pathway therapy for urea cycle disorders: twenty years later". The Journal of Pediatrics. 138 (1 Suppl): S46–S54, discussion S54–S55. doi:10.1067/mpd.2001.111836. PMID 11148549.
^Walker V (September 2009). "Ammonia toxicity and its prevention in inherited defects of the urea cycle". Diabetes, Obesity & Metabolism. 11 (9): 823–835. doi:10.1111/j.1463-1326.2009.01054.x. PMID 19531057. S2CID 25998574.
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