No edit summary |
83.233.36.201 (talk) No edit summary |
||
Line 28: | Line 28: | ||
| routes_of_administration = |
| routes_of_administration = |
||
}} |
}} |
||
'''Sodium phenylbutyrate''' is an [[orphan drug]], marketed by [[Ucyclyd Pharma]] ([[Hunt Valley]], USA) under the trade name '''Buphenyl®''' and by Orphan |
'''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 disorder]]s.<ref>{{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=}}</ref> |
It has been used to treat [[urea cycle disorder]]s.<ref>{{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=}}</ref> |
Revision as of 07:45, 20 March 2008
![]() | |
Clinical data | |
---|---|
License data |
|
Pregnancy category |
|
ATC code | |
Pharmacokinetic data | |
Metabolism | to phenylacetate |
Elimination half-life | 0.8 hours |
Excretion | 80% as phenylacetylglutamine |
Identifiers | |
| |
CAS Number | |
PubChem CID | |
CompTox Dashboard (EPA) | |
ECHA InfoCard | 100.130.318 |
Chemical and physical data | |
Formula | C10H11NaO2 |
Molar mass | 186.2 g/mol g·mol−1 |
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]
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.
![](https://upload.wikimedia.org/wikipedia/commons/f/fd/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.[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.
{{cite journal}}
: CS1 maint: multiple names: authors list (link) - ^ 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.
{{cite journal}}
: CS1 maint: multiple names: authors list (link)