Glycerol phenylbutyrate | HPN-100 | CAS#611168-24-2

MedKoo Cat#: 319730 | Name: Glycerol phenylbutyrate

Description:

WARNING: This product is for research use only, not for human or veterinary use.

Glycerol phenylbutyrate, also known as HPN-100, is under trials in the treatment of certain inborn urea cycle disorders. The medication works by preventing the harmful buildup of ammonia in the body. Ammonia is a neurotoxic agent that is primarily generated in the intestine and detoxified in the liver. Toxic increases in systemic ammonia levels predominantly result from an inherited or acquired impairment in hepatic detoxification and lead to potentially life-threatening neuropsychiatric symptoms.

Chemical Structure

Glycerol phenylbutyrate

CAS#611168-24-2

Theoretical Analysis

MedKoo Cat#: 319730

Name: Glycerol phenylbutyrate

CAS#: 611168-24-2

Chemical Formula: C33H38O6

Exact Mass: 530.2700

Molecular Weight: 530.66

Elemental Analysis: C, 74.69; H, 7.22; O, 18.09

Price and Availability

Size	Price	Availability
10mg	USD 85.00	Ready to ship
50mg	USD 150.00	Ready to ship
100mg	USD 250.00	Ready to ship
200mg	USD 400.00	Ready to ship
500mg	USD 650.00	Ready to ship
1g	USD 1,050.00	Ready to ship

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Related CAS #

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Synonym

HPN-100; HPN 100; HPN100; Glycerol phenylbutyrate, trade name: Ravicti.

IUPAC/Chemical Name

propane-1,2,3-triyl tris(4-phenylbutanoate)

InChi Key

ZSDBFLMJVAGKOU-UHFFFAOYSA-N

InChi Code

InChI=1S/C33H38O6/c34-31(22-10-19-27-13-4-1-5-14-27)37-25-30(39-33(36)24-12-21-29-17-8-3-9-18-29)26-38-32(35)23-11-20-28-15-6-2-7-16-28/h1-9,13-18,30H,10-12,19-26H2

SMILES Code

O=C(OCC(OC(CCCC1=CC=CC=C1)=O)COC(CCCC2=CC=CC=C2)=O)CCCC3=CC=CC=C3

Appearance

Colorless to light yellow oil

Purity

>98% (or refer to the Certificate of Analysis)

Shipping Condition

Shipped under ambient temperature as non-hazardous chemical. This product is stable enough for a few weeks during ordinary shipping and time spent in Customs.

Storage Condition

Dry, dark and at 0 - 4 C for short term (days to weeks) or -20 C for long term (months to years).

Solubility

Soluble in DMSO and ethanol

Shelf Life

>2 years if stored properly

Drug Formulation

This drug may be formulated in DMSO and ethanol

Stock Solution Storage

0 - 4 C for short term (days to weeks), or -20 C for long term (months).

HS Tariff Code

2934.99.9001

More Info

Product Data

Product Data

Certificate of Analysis

View CoA: current batch, Lot#S23S03C27

QC Data

View QC data: current batch, Lot#S23S03C27

Safety Data Sheet (SDS)

View Safety Data Sheet (SDS)

Instruction

View handling instruction

Biological target:

Glycerol phenylbutyrate is a sigma-2 (σ2) receptor ligand with a pKi of 8.02.

In vitro activity:

To be determined

In vivo activity:

GPB dosed at 6 mL twice daily lowered fasting ammonia levels in cirrhotic patients with hepatic encephalopathy as compared with baseline, was better tolerated than 9 mL twice daily, and is appropriate for further evaluation in patients with cirrhosis and episodic hepatic encephalopathy. Reference: Clin Pharmacol Drug Dev. 2013 Jul;2(3):278-84. https://pubmed.ncbi.nlm.nih.gov/27121790/

	Solvent	mg/mL	mM
Solubility
	DMSO	150.0	282.67
	Ethanol	50.0	94.22

Note: There can be variations in solubility for the same chemical from different vendors or different batches from the same vendor. The following factors can affect the solubility of the same chemical: solvent used for crystallization, residual solvent content, polymorphism, salt versus free form, degree of hydration, solvent temperature. Please use the solubility data as a reference only. Warming and sonication will facilitate dissolving. Still have questions? Please contact our Technical Support scientists.

Preparing Stock Solutions

The following data is based on the product molecular weight 530.66 Batch specific molecular weights may vary from batch to batch due to the degree of hydration, which will affect the solvent volumes required to prepare stock solutions.

Concentration / Solvent Volume / Mass	1 mg	5 mg	10 mg
1 mM	1.15 mL	5.76 mL	11.51 mL
5 mM	0.23 mL	1.15 mL	2.3 mL
10 mM	0.12 mL	0.58 mL	1.15 mL
50 mM	0.02 mL	0.12 mL	0.23 mL

Formulation protocol:

1. Ghabril M, Zupanets IA, Vierling J, Mantry P, Rockey D, Wolf D, O'Shea R, Dickinson K, Gillaspy H, Norris C, Coakley DF, Mokhtarani M, Scharschmidt BF. Glycerol Phenylbutyrate in Patients With Cirrhosis and Episodic Hepatic Encephalopathy: A Pilot Study of Safety and Effect on Venous Ammonia Concentration. Clin Pharmacol Drug Dev. 2013 Jul;2(3):278-84. doi: 10.1002/cpdd.18. Epub 2013 Mar 16. PMID: 27121790. 2. Smith W, Diaz GA, Lichter-Konecki U, Berry SA, Harding CO, McCandless SE, LeMons C, Mauney J, Dickinson K, Coakley DF, Moors T, Mokhtarani M, Scharschmidt BF, Lee B. Ammonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate. J Pediatr. 2013 Jun;162(6):1228-34, 1234.e1. doi: 10.1016/j.jpeds.2012.11.084. Epub 2013 Jan 13. PMID: 23324524; PMCID: PMC4017326.

In vitro protocol:

To be determined

In vivo protocol:

1: Nagamani SC, Diaz GA, Rhead W, Berry SA, Le Mons C, Lichter-Konecki U, Bartley J, Feigenbaum A, Schulze A, Longo N, Berquist W, Gallagher R, Bartholomew D, Harding CO, Korson MS, McCandless SE, Smith W, Vockley J, Kronn D, Zori R, Cederbaum S, Merritt JL 2nd, Wong D, Coakley DF, Scharschmidt BF, Dickinson K, Marino M, Lee BH, Mokhtarani M. Self-reported treatment-associated symptoms among patients with urea cycle disorders participating in glycerol phenylbutyrate clinical trials. Mol Genet Metab. 2015 Sep-Oct;116(1-2):29-34. doi: 10.1016/j.ymgme.2015.08.002. Epub 2015 Aug 5. PubMed PMID: 26296711. 2: Glycerol phenylbutyrate (Ravicti) for urea cycle disorders. Med Lett Drugs Ther. 2014 Aug 18;56(1449):77-8. Review. PubMed PMID: 25118801. 3: Berry SA, Lichter-Konecki U, Diaz GA, McCandless SE, Rhead W, Smith W, Lemons C, Nagamani SC, Coakley DF, Mokhtarani M, Scharschmidt BF, Lee B. Glycerol phenylbutyrate treatment in children with urea cycle disorders: pooled analysis of short and long-term ammonia control and outcomes. Mol Genet Metab. 2014 May;112(1):17-24. doi: 10.1016/j.ymgme.2014.02.007. Epub 2014 Feb 21. PubMed PMID: 24630270; PubMed Central PMCID: PMC4382922. 4: Rockey DC, Vierling JM, Mantry P, Ghabril M, Brown RS Jr, Alexeeva O, Zupanets IA, Grinevich V, Baranovsky A, Dudar L, Fadieienko G, Kharchenko N, Klaryts'ka I, Morozov V, Grewal P, McCashland T, Reddy KG, Reddy KR, Syplyviy V, Bass NM, Dickinson K, Norris C, Coakley D, Mokhtarani M, Scharschmidt BF; HALT-HE Study Group. Randomized, double-blind, controlled study of glycerol phenylbutyrate in hepatic encephalopathy. Hepatology. 2014 Mar;59(3):1073-83. doi: 10.1002/hep.26611. PubMed PMID: 23847109; PubMed Central PMCID: PMC4237123. 5: Monteleone JP, Mokhtarani M, Diaz GA, Rhead W, Lichter-Konecki U, Berry SA, Lemons C, Dickinson K, Coakley D, Lee B, Scharschmidt BF. Population pharmacokinetic modeling and dosing simulations of nitrogen-scavenging compounds: disposition of glycerol phenylbutyrate and sodium phenylbutyrate in adult and pediatric patients with urea cycle disorders. J Clin Pharmacol. 2013 Jul;53(7):699-710. doi: 10.1002/jcph.92. Epub 2013 Jun 15. PubMed PMID: 23775211; PubMed Central PMCID: PMC3923458. 6: Smith W, Diaz GA, Lichter-Konecki U, Berry SA, Harding CO, McCandless SE, LeMons C, Mauney J, Dickinson K, Coakley DF, Moors T, Mokhtarani M, Scharschmidt BF, Lee B. Ammonia control in children ages 2 months through 5 years with urea cycle disorders: comparison of sodium phenylbutyrate and glycerol phenylbutyrate. J Pediatr. 2013 Jun;162(6):1228-34, 1234.e1. doi: 10.1016/j.jpeds.2012.11.084. Epub 2013 Jan 13. PubMed PMID: 23324524; PubMed Central PMCID: PMC4017326. 7: Diaz GA, Krivitzky LS, Mokhtarani M, Rhead W, Bartley J, Feigenbaum A, Longo N, Berquist W, Berry SA, Gallagher R, Lichter-Konecki U, Bartholomew D, Harding CO, Cederbaum S, McCandless SE, Smith W, Vockley G, Bart SA, Korson MS, Kronn D, Zori R, Merritt JL 2nd, C S Nagamani S, Mauney J, Lemons C, Dickinson K, Moors TL, Coakley DF, Scharschmidt BF, Lee B. Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate. Hepatology. 2013 Jun;57(6):2171-9. doi: 10.1002/hep.26058. Epub 2013 Jan 3. PubMed PMID: 22961727; PubMed Central PMCID: PMC3557606. 8: Lichter-Konecki U, Diaz GA, Merritt JL 2nd, Feigenbaum A, Jomphe C, Marier JF, Beliveau M, Mauney J, Dickinson K, Martinez A, Mokhtarani M, Scharschmidt B, Rhead W. Ammonia control in children with urea cycle disorders (UCDs); phase 2 comparison of sodium phenylbutyrate and glycerol phenylbutyrate. Mol Genet Metab. 2011 Aug;103(4):323-9. doi: 10.1016/j.ymgme.2011.04.013. Epub 2011 May 5. PubMed PMID: 21612962. 9: McGuire BM, Zupanets IA, Lowe ME, Xiao X, Syplyviy VA, Monteleone J, Gargosky S, Dickinson K, Martinez A, Mokhtarani M, Scharschmidt BF. Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis. Hepatology. 2010 Jun;51(6):2077-85. doi: 10.1002/hep.23589. PubMed PMID: 20512995; PubMed Central PMCID: PMC3733097.