- 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 byUcyclyd 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 disorder s. [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 bybeta-oxidation tophenylacetate .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 malignantglioma andacute 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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