Sodium phenylbutyrate

Sodium phenylbutyrate

drugbox
IUPAC_name = Sodium 4-phenylbutanoate



CAS_number = 1716-12-7
ATC_prefix = A16
ATC_suffix = AX03
PubChem = 15585
DrugBank =
C = 10
H = 11
O = 2
Na = 1
molecular_weight = 186.2 g/mol
bioavailability =
protein_bound =
metabolism = to phenylacetate
elimination_half-life = 0.8 hours
excretion = 80% as phenylacetylglutamine
licence_EU = Ammonaps
licence_US = Sodium_phenylbutyrate
pregnancy_AU =
pregnancy_US =
pregnancy_category = not to be used
legal_AU =
legal_UK =
legal_US =
legal_status =
routes_of_administration =

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. [cite journal |author=Batshaw ML, MacArthur RB, Tuchman M |title=Alternative pathway therapy for urea cycle disorders: twenty years later |journal=J. Pediatr. |volume=138 |issue=1 Suppl |pages=S46–54; discussion S54–5 |year=2001 |pmid=11148549 |doi=]

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. [cite journal |author=Kang HL, Benzer S, Min KT |title=Life extension in Drosophila by feeding a drug |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue=2 |pages=838–43 |year=2002 |pmid=11792861 |doi=10.1073/pnas.022631999]

References


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