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1. Hpn 100
2. Hpn-100
3. Hpn100
4. Ravicti
1. 611168-24-2
2. Ravicti
3. Hpn-100
4. Glycerolphenylbutyrate
5. Gt4p
6. Hpn100
7. Propane-1,2,3-triyl Tris(4-phenylbutanoate)
8. Zh6f1vcv7b
9. Glycerol Phenylbutyrate [usan]
10. Glycerol Phenylbutyrate (usan)
11. Benzenebutanoic Acid, 1,1',1''-(1,2,3-propanetriyl) Ester
12. Unii-zh6f1vcv7b
13. Tris(4-phenylbutyryl)glycerol
14. Glyceryl Tri-4-phenylbutyrate
15. Glyceryl Tri-(4-phenylbutyrate)
16. Hpn 100
17. Glycerol Phenylbutyrate [usan:inn]
18. Ravicti (tn)
19. Glycerol-phenylbutyrate
20. 2,3-bis(4-phenylbutanoyloxy)propyl 4-phenylbutanoate
21. Chembl2105745
22. Schembl10102804
23. Dtxsid40210005
24. Hpn-100hpn-100
25. Chebi:134745
26. Hy-b2087
27. Glycerol Phenylbutyrate [mi]
28. Tri(4-phenylbutyryl)glycerol
29. Glycerol Phenylbutyrate [inn]
30. S6981
31. Glyceryl Tri(4-phenylbutyrate)
32. At33615
33. Db08909
34. Glycerol Phenylbutyrate [vandf]
35. Glycerol Phenylbutyrate [who-dd]
36. Glyceryl Tri (4-phenylbutyrate)
37. Cs-0017499
38. Glycerol Phenylbutyrate [orange Book]
39. D10127
40. Q15322709
41. Benzenebutanoic Acid, 1,2,3-propanetriyl Ester
Molecular Weight | 530.6 g/mol |
---|---|
Molecular Formula | C33H38O6 |
XLogP3 | 6.7 |
Hydrogen Bond Donor Count | 0 |
Hydrogen Bond Acceptor Count | 6 |
Rotatable Bond Count | 20 |
Exact Mass | 530.26683893 g/mol |
Monoisotopic Mass | 530.26683893 g/mol |
Topological Polar Surface Area | 78.9 Ų |
Heavy Atom Count | 39 |
Formal Charge | 0 |
Complexity | 648 |
Isotope Atom Count | 0 |
Defined Atom Stereocenter Count | 0 |
Undefined Atom Stereocenter Count | 0 |
Defined Bond Stereocenter Count | 0 |
Undefined Bond Stereocenter Count | 0 |
Covalently Bonded Unit Count | 1 |
1 of 2 | |
---|---|
Drug Name | Ravicti |
PubMed Health | Glycerol Phenylbutyrate (By mouth) |
Drug Classes | Hyperammonemia Agent |
Drug Label | RAVICTI (glycerol phenylbutyrate) is a clear, colorless to pale yellow oral liquid. It is insoluble in water and most organic solvents, and it is soluble in dimethylsulfoxide (DMSO) and >65% acetonitrile.Glycerol phenylbutyrate is a nitrogen-binding... |
Active Ingredient | Glycerol phenylbutyrate |
Dosage Form | Liquid |
Route | Oral |
Strength | 1.1gm/ml |
Market Status | Prescription |
Company | Hyperion Therap |
2 of 2 | |
---|---|
Drug Name | Ravicti |
PubMed Health | Glycerol Phenylbutyrate (By mouth) |
Drug Classes | Hyperammonemia Agent |
Drug Label | RAVICTI (glycerol phenylbutyrate) is a clear, colorless to pale yellow oral liquid. It is insoluble in water and most organic solvents, and it is soluble in dimethylsulfoxide (DMSO) and >65% acetonitrile.Glycerol phenylbutyrate is a nitrogen-binding... |
Active Ingredient | Glycerol phenylbutyrate |
Dosage Form | Liquid |
Route | Oral |
Strength | 1.1gm/ml |
Market Status | Prescription |
Company | Hyperion Therap |
Glycerol phenylbutyrate is a nitrogen-binding agent for the chronic management of adult and pediatric patients 2 years of age with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.
FDA Label
Ravicti is indicated for use as adjunctive therapy for chronic management of patients with urea cycle disorders (UCDs) including deficiencies of carbamoyl phosphate-synthase-I (CPS), ornithine carbamoyltransferase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase I (ARG) and ornithine translocase deficiency hyperornithinaemia-hyperammonaemia homocitrullinuria syndrome (HHH) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.
Ravicti must be used with dietary protein restriction and, in some cases, dietary supplements (e. g. , essential amino acids, arginine, citrulline, protein-free calorie supplements).
Glycerol phenylbutyrate prolongs the QTc interval.
A16AX09
A - Alimentary tract and metabolism
A16 - Other alimentary tract and metabolism products
A16A - Other alimentary tract and metabolism products
A16AX - Various alimentary tract and metabolism products
A16AX09 - Glycerol phenylbutyrate
Absorption
Glycerol phenylbutyrate is a prodrug in which phenylbutyrate (PBA) is released from the glycerol backbone by lipases in the gastrointestinal tract. PBA then undergoes beta-oxidtion to form PAA. When a single oral dose of 2.9 mL/m2 of Glycerol phenylbutyrate is given to fasting adult subjects, the pharmacokinetic parameters are as follows: Tmax: PBA = 2 hours; PAA = 4 hours; PAGN = 4 hours. Cmax: PBA = 37.0 g/mL; PAA = 14.9 g/mL; PAGN = 30.2 g/mL. In healthy subjects, the hydrolysis of glycerol phenylbutyrate is incomplete, but to what extent is unknown. When glycerol phenylbutyrate is given to adult UCD patients, maximum plasma concentrations at steady state (Cmaxss) of PBA, PAA, and PAGN occurred at 8 h, 12 h, and 10 h, respectively, after the first dose in the day. Intact glycerol phenylbutyrate was not detectable in plasma in UCD patients.
Route of Elimination
Glycerol phenylbutyrate is mainly excreted as PAGN in the urine (68.9% in adults and 66.5% in pediatric UCD patients). PAA and PBA represented minor urinary metabolites, each accounting for <1% of the administered dose of PBA.
Pancreatic lipases hydrolyze glycerol phenylbutyrate to release PBA from the glycerol backbone. PBA undergoes -oxidation to PAA, which is conjugated with glutamine in the liver and in the kidney through the enzyme phenylacetyl-CoA: L-glutamine-N-acetyltransferase to form PAGN.
The toxic accumulation of ammonia in the blood and brain arise from urea cycle disorders in which patients are deficient in critical enzymes or transporters that are involved in the synthesis of urea from ammonia. Glycerol phenylbutyrate is a prodrug - the major metabolite, phenylacetate (PAA) is the molecule that binds to nitrogen. PAA conjugates with glutamine (which contains 2 molecules of nitrogen) via acetylation in the liver and kidneys to form phenylacetylglutamine (PAGN), which is excreted by the kidneys. PAGN, like urea, contains 2 moles of nitrogen and provides an alternate vehicle for waste nitrogen excretion.
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PharmaCompass offers a list of Glycerol Phenylbutyrate API manufacturers, exporters & distributors, which can be sorted by GMP, USDMF, JDMF, KDMF, CEP (COS), WC, Price,and more, enabling you to easily find the right Glycerol Phenylbutyrate manufacturer or Glycerol Phenylbutyrate supplier for your needs.
Send us enquiries for free, and we will assist you in establishing a direct connection with your preferred Glycerol Phenylbutyrate manufacturer or Glycerol Phenylbutyrate supplier.
PharmaCompass also assists you with knowing the Glycerol Phenylbutyrate API Price utilized in the formulation of products. Glycerol Phenylbutyrate API Price is not always fixed or binding as the Glycerol Phenylbutyrate Price is obtained through a variety of data sources. The Glycerol Phenylbutyrate Price can also vary due to multiple factors, including market conditions, regulatory modifications, or negotiated pricing deals.
A 611168-24-2 manufacturer is defined as any person or entity involved in the manufacture, preparation, processing, compounding or propagation of 611168-24-2, including repackagers and relabelers. The FDA regulates 611168-24-2 manufacturers to ensure that their products comply with relevant laws and regulations and are safe and effective to use. 611168-24-2 API Manufacturers are required to adhere to Good Manufacturing Practices (GMP) to ensure that their products are consistently manufactured to meet established quality criteria.
click here to find a list of 611168-24-2 manufacturers with USDMF, JDMF, KDMF, CEP, GMP, COA and API Price related information on PhamaCompass.
A 611168-24-2 supplier is an individual or a company that provides 611168-24-2 active pharmaceutical ingredient (API) or 611168-24-2 finished formulations upon request. The 611168-24-2 suppliers may include 611168-24-2 API manufacturers, exporters, distributors and traders.
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A 611168-24-2 DMF (Drug Master File) is a document detailing the whole manufacturing process of 611168-24-2 active pharmaceutical ingredient (API) in detail. Different forms of 611168-24-2 DMFs exist exist since differing nations have different regulations, such as 611168-24-2 USDMF, ASMF (EDMF), JDMF, CDMF, etc.
A 611168-24-2 DMF submitted to regulatory agencies in the US is known as a USDMF. 611168-24-2 USDMF includes data on 611168-24-2's chemical properties, information on the facilities and procedures used, and details about packaging and storage. The 611168-24-2 USDMF is kept confidential to protect the manufacturer’s intellectual property.
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A 611168-24-2 written confirmation (611168-24-2 WC) is an official document issued by a regulatory agency to a 611168-24-2 manufacturer, verifying that the manufacturing facility of a 611168-24-2 active pharmaceutical ingredient (API) adheres to the Good Manufacturing Practices (GMP) regulations of the importing country. When exporting 611168-24-2 APIs or 611168-24-2 finished pharmaceutical products to another nation, regulatory agencies frequently require a 611168-24-2 WC (written confirmation) as part of the regulatory process.
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National Drug Code is a comprehensive database maintained by the FDA that contains information on all drugs marketed in the US. This directory includes information about finished drug products, unfinished drug products, and compounded drug products, including those containing 611168-24-2 as an active pharmaceutical ingredient (API).
The FDA updates the NDC directory daily. The NDC numbers for 611168-24-2 API and other APIs are published in this directory by the FDA.
The NDC unfinished drugs database includes product listing information submitted for all unfinished drugs, such as active pharmaceutical ingredients (APIs), drugs intended for further processing and bulk drug substances for compounding.
Pharmaceutical companies that manufacture 611168-24-2 as an active pharmaceutical ingredient (API) must furnish the FDA with an updated record of all drugs that they produce, prepare, propagate, compound, or process for commercial distribution in the US at their facilities.
The NDC directory also contains data on finished compounded human drug products that contain 611168-24-2 and are produced by outsourcing facilities. While these outsourcing facilities are not mandated to assign a 611168-24-2 NDC to their finished compounded human drug products, they may choose to do so.
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611168-24-2 Active pharmaceutical ingredient (API) is produced in GMP-certified manufacturing facility.
GMP stands for Good Manufacturing Practices, which is a system used in the pharmaceutical industry to make sure that goods are regularly produced and monitored in accordance with quality standards. The FDA’s current Good Manufacturing Practices requirements are referred to as cGMP or current GMP which indicates that the company follows the most recent GMP specifications. The World Health Organization (WHO) has its own set of GMP guidelines, called the WHO GMP. Different countries can also set their own guidelines for GMP like China (Chinese GMP) or the EU (EU GMP).
PharmaCompass offers a list of 611168-24-2 GMP manufacturers, exporters & distributors, which can be sorted by USDMF, JDMF, KDMF, CEP (COS), WC, API price, and more, enabling you to easily find the right 611168-24-2 GMP manufacturer or 611168-24-2 GMP API supplier for your needs.
A 611168-24-2 CoA (Certificate of Analysis) is a formal document that attests to 611168-24-2's compliance with 611168-24-2 specifications and serves as a tool for batch-level quality control.
611168-24-2 CoA mostly includes findings from lab analyses of a specific batch. For each 611168-24-2 CoA document that a company creates, the USFDA specifies specific requirements, such as supplier information, material identification, transportation data, evidence of conformity and signature data.
611168-24-2 may be tested according to a variety of international standards, such as European Pharmacopoeia (611168-24-2 EP), 611168-24-2 JP (Japanese Pharmacopeia) and the US Pharmacopoeia (611168-24-2 USP).