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'''Sodium phenylbutyrate''' is an [[orphan drug]], marketed by [[Ucyclyd Pharma]] ([[Hunt Valley]], USA) under the trade name '''Buphenyl |
'''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'''. |
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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> |
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Phenylacetate conjugate with glutamine to [[phenylacetylglutamine]], that is eliminated with the urine. |
Phenylacetate conjugate with glutamine to [[phenylacetylglutamine]], that is eliminated with the urine. |
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==Research== |
==Research== |
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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]]. |
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]]. |
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PBA has been associated with longer lifespans in [[Drosophila]].<ref>{{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 |
PBA has been associated with longer lifespans in [[Drosophila]].<ref>{{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}}</ref> |
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==References == |
==References == |
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{{Other alimentary tract and metabolism products}} |
{{Other alimentary tract and metabolism products}} |
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[[Category:Orphan drugs]] |
[[Category:Orphan drugs]] |
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[[Category:Prodrugs]] |
[[Category:Prodrugs]] |
Revision as of 18:04, 25 April 2008
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Clinical data | |
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License data |
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Pregnancy category |
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ATC code | |
Pharmacokinetic data | |
Metabolism | to phenylacetate |
Elimination half-life | 0.8 hours |
Excretion | 80% as phenylacetylglutamine |
Identifiers | |
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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)