GLYCEROL PHENYLBUTYRATE

Details

Stereochemistry	ACHIRAL
Molecular Formula	C33H38O6
Molecular Weight	530.6512
Optical Activity	NONE
Defined Stereocenters	0 / 0
E/Z Centers	0
Charge	0

SHOW SMILES / InChI

Structure Search

SMILES

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

InChI

InChIKey=ZSDBFLMJVAGKOU-UHFFFAOYSA-N

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

HIDE SMILES / InChI

Description
Sources: http://www.accessdata.fda.gov/drugsatfda_docs/label/2009/020572s016,020573s015lbl.pdf

Sodium phenylbutyrate is a salt of an aromatic fatty acid. The compound is used to treat urea cycle disorders, because its metabolites offer an alternative pathway to the urea cycle to allow excretion of excess nitrogen. Sodium phenylbutyrate is also a histone deacetylase inhibitor and chemical chaperone, leading respectively to research into its use as an anti-cancer agent and in protein misfolding diseases such as cystic fibrosis. It is used as adjunctive therapy for the management of chronic urea cycle disorders due to deficiencies in carbamylphosphate (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase. It is indicated in all neonatal- onset efficiency presenting within the first 28 days of life. Also indicated in patients with late-onset, presenting after the first month of life with a history of hyperammonemic encephalopathy. Sodium phenylbutyrate is a pro-drug and is rapidly metabolized to phenylacetate. Phenylacetate is a metabolically active compound that conjugates with glutamine via acetylation to form phenylacetylglutamine. The kidneys then excrete Phenylacetylglutamine. PBA (phenylbutyric acid) is absorbed from the intestine and converted by way of β-oxidation to the active moiety, phenylacetic acid (PAA). PAA is conjugated with glutamine in the liver and kidney by way of N-acyl coenzyme A-l-glutamine N-acyltransferase to form phenylacetylglutamine (PAGN). Like urea, PAGN incorporates two waste nitrogens and is excreted in the urine. On a molar basis, it is comparable to urea (each containing two moles of nitrogen). Therefore, phenylacetylglutamine provides an alternate vehicle for waste nitrogen excretion.

CNS Activity

Approval Year

Targets

Primary Target	Pharmacology	Potency
cell cycle arrest 14 Target ID: GO:0007050 Sources: https://www.ncbi.nlm.nih.gov/pubmed/19024104	Activator 1430
Histone deacetylase 23 Target ID: CHEMBL2093865 Sources: https://www.ncbi.nlm.nih.gov/pubmed/17164759\|https://www.ncbi.nlm.nih.gov/pubmed/21894430\|https://www.ncbi.nlm.nih.gov/pubmed/15626823\|https://www.ncbi.nlm.nih.gov/pubmed/19520580	Inhibitor 8297	6.34 µM [Ki]
Glutamine 3 Target ID: CHEMBL2366039 Sources: https://www.ncbi.nlm.nih.gov/pubmed/20512995	Binding Agent 1064
Nitrogen 3 Target ID: Nitrogen Sources: https://www.genome.jp/dbget-bin/www_bget?dr:D10127 \| https://www.ncbi.nlm.nih.gov/pubmed/29381293	Binding Agent 1064

Conditions

Condition	Modality	Targets	Highest Phase	Product
Urea cycle disorders 3 Sources: http://www.accessdata.fda.gov/drugsatfda_docs/label/2009/020572s016,020573s015lbl.pdf	Palliative		Approved 8429	BUPHENYL Approved Use BUPHENYL® is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS). It is indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzymatic deficiency, presenting after the first month of life) who have a history of hyperammonemic encephalopathy. It is important that the diagnosis be made early and treatment initiated immediately to improve survival. Any episode of acute hyperammonemia should be treated as a lifethreatening emergency. Launch Date 1996
Late-onset disease 3 Sources: http://www.accessdata.fda.gov/drugsatfda_docs/label/2009/020572s016,020573s015lbl.pdf	Palliative		Approved 8429	BUPHENYL Approved Use BUPHENYL® is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS). It is indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzymatic deficiency, presenting after the first month of life) who have a history of hyperammonemic encephalopathy. It is important that the diagnosis be made early and treatment initiated immediately to improve survival. Any episode of acute hyperammonemia should be treated as a lifethreatening emergency. Launch Date 1996

C_max

Value	Dose	Analyte	Population
949.9 μM EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	18 g 1 times / day multiple, oral dose: 18 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
1679.8 μM EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	27 g 1 times / day multiple, oral dose: 27 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
1878.3 μM EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	36 g 1 times / day multiple, oral dose: 36 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
1669.4 μM EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	45 g 1 times / day multiple, oral dose: 45 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
457.9 μM EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	9 g 1 times / day multiple, oral dose: 9 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
218 μg/mL DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2009/020572s016,020573s015lbl.pdf	5 mg single, oral dose: 5 mg route of administration: Oral experiment type: SINGLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: HEALTHY age: ADULT sex: UNKNOWN food status: FASTED

AUC

Value	Dose	Analyte	Population
2170.6 μM × h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	18 g 1 times / day multiple, oral dose: 18 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
3778.6 μM × h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	27 g 1 times / day multiple, oral dose: 27 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
4687.4 μM × h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	36 g 1 times / day multiple, oral dose: 36 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
4941.4 μM × h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	45 g 1 times / day multiple, oral dose: 45 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
925.5 μM × h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	9 g 1 times / day multiple, oral dose: 9 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED

T_1/2

Value	Dose	Analyte	Population
0.61 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	18 g 1 times / day multiple, oral dose: 18 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
0.58 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	27 g 1 times / day multiple, oral dose: 27 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
0.94 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	36 g 1 times / day multiple, oral dose: 36 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
1.16 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	45 g 1 times / day multiple, oral dose: 45 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
0.76 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/11489804	9 g 1 times / day multiple, oral dose: 9 g route of administration: Oral experiment type: MULTIPLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNHEALTHY age: ADULT sex: FEMALE / MALE food status: FED
0.77 h DRUG LABEL https://www.accessdata.fda.gov/drugsatfda_docs/label/2009/020572s016,020573s015lbl.pdf	5 mg single, oral dose: 5 mg route of administration: Oral experiment type: SINGLE co-administered:	SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: HEALTHY age: ADULT sex: UNKNOWN food status: FASTED

F_unbound

Value	Dose	Co-administered	Analyte	Population
0.3% EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/8946671	unknown, unknown		SODIUM PHENYLBUTYRATE plasma	Homo sapiens population: UNKNOWN age: UNKNOWN sex: UNKNOWN food status: UNKNOWN

Doses

Dose	Population	Adverse events
18 g 1 times / day steady, oral Dose: 18 g, 1 times / day Route: oral Route: steady Dose: 18 g, 1 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/17702032	unhealthy, 31-73 years n = 11 Health Status: unhealthy Condition: Huntington’s Disease Age Group: 31-73 years Sex: M+F Population Size: 11 Sources: https://pubmed.ncbi.nlm.nih.gov/17702032	DLT: Nausea, Vomiting... Dose limiting toxicities: Nausea (grade 3, 2 patients) Vomiting (grade 3, 1 patient) Gait instability (grade 3, 2 patients) Lightheadedness (grade 3, 2 patients) Confusion (grade 3, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/17702032
15 g 1 times / day steady, oral MTD Dose: 15 g, 1 times / day Route: oral Route: steady Dose: 15 g, 1 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/17702032	unhealthy, 38-61 years n = 4 Health Status: unhealthy Condition: Huntington’s Disease Age Group: 38-61 years Sex: M+F Population Size: 4 Sources: https://pubmed.ncbi.nlm.nih.gov/17702032	Other AEs: Nausea, Gait instability... Other AEs: Nausea (grade 1, 2 patients) Gait instability (grade 1, 1 patient) Syncope vasovagal (grade 2, 2 patients) Diarrhea (grade 1, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/17702032
27 g 1 times / day steady, oral MTD Dose: 27 g, 1 times / day Route: oral Route: steady Dose: 27 g, 1 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/15831235	unhealthy, 51.4 years (range: 29.7–71.1 years) n = 7 Health Status: unhealthy Condition: recurrent malignant gliomas Age Group: 51.4 years (range: 29.7–71.1 years) Sex: M+F Population Size: 7 Sources: https://pubmed.ncbi.nlm.nih.gov/15831235	DLT: Fatigue... Dose limiting toxicities: Fatigue (grade 3, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/15831235
36 g 1 times / day steady, oral Dose: 36 g, 1 times / day Route: oral Route: steady Dose: 36 g, 1 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/15831235	unhealthy, 51.4 years (range: 29.7–71.1 years) n = 4 Health Status: unhealthy Condition: recurrent malignant gliomas Age Group: 51.4 years (range: 29.7–71.1 years) Sex: M+F Population Size: 4 Sources: https://pubmed.ncbi.nlm.nih.gov/15831235	DLT: Fatigue, Somnolence... Dose limiting toxicities: Fatigue (grade 3, 2 patients) Somnolence (grade 3, 2 patients) Anemia (grade 3, 1 patient) Neutropenia (grade 3, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/15831235
45 g single, intravenous Highest studied dose Dose: 45 g Route: intravenous Route: single Dose: 45 g Sources: https://pubmed.ncbi.nlm.nih.gov/11489804	unhealthy, 57 years (range: 26–75 years) n = 4 Health Status: unhealthy Age Group: 57 years (range: 26–75 years) Sex: M+F Population Size: 4 Sources: https://pubmed.ncbi.nlm.nih.gov/11489804	Sources: https://pubmed.ncbi.nlm.nih.gov/11489804
45 g 1 times / day steady, oral Highest studied dose Dose: 45 g, 1 times / day Route: oral Route: steady Dose: 45 g, 1 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/11489804	unhealthy, 57 years (range: 26–75 years) n = 4 Health Status: unhealthy Age Group: 57 years (range: 26–75 years) Sex: M+F Population Size: 4 Sources: https://pubmed.ncbi.nlm.nih.gov/11489804	DLT: Fatigue, Central nervous system toxicity... Other AEs: Nausea, Vomiting... Dose limiting toxicities: Fatigue (grade 3-4, 1 patient) Central nervous system toxicity (grade 3-4, 1 patient) Other AEs: Nausea (grade 3-4, 1 patient) Vomiting (grade 3-4, 1 patient) Edema (grade 3-4, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/11489804
36 g 1 times / day steady, oral Dose: 36 g, 1 times / day Route: oral Route: steady Dose: 36 g, 1 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/11489804	unhealthy, 57 years (range: 26–75 years) n = 7 Health Status: unhealthy Age Group: 57 years (range: 26–75 years) Sex: M+F Population Size: 7 Sources: https://pubmed.ncbi.nlm.nih.gov/11489804	DLT: Nausea, Vomiting... Dose limiting toxicities: Nausea (grade 3-4, 1 patient) Vomiting (grade 3-4, 1 patient) Hypocalcemia (grade 3-4, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/11489804
150 mg/kg 3 times / day steady, oral MTD Dose: 150 mg/kg, 3 times / day Route: oral Route: steady Dose: 150 mg/kg, 3 times / day Sources: https://pubmed.ncbi.nlm.nih.gov/17053987	unhealthy, 57 years(range: 25–71 years) n = 3 Health Status: unhealthy Age Group: 57 years(range: 25–71 years) Sex: M+F Population Size: 3 Sources: https://pubmed.ncbi.nlm.nih.gov/17053987	DLT: Fatigue... Other AEs: Anemia, Light-headed... Dose limiting toxicities: Fatigue (grade 2, 2 patients) Other AEs: Anemia (grade 3, 1 patient) Light-headed (grade 2, 2 patients) Edema (grade 2, 2 patients) Nausea (grade 2, 1 patient) Vomiting (grade 2, 1 patient) Constipation (grade 1, 2 patients) Fever (grade 1, 1 patient) Sources: https://pubmed.ncbi.nlm.nih.gov/17053987

AEs

Sourcing

Vendor/Aggregator	ID	URL
001 Chemical	DY533818	https://www.001chemical.com/chem/1821-12-1
3B Scientific (Wuhan) Corp	3B3-017601	http://www.3bsc.com/index/pro_info.php?id=40125
AA Blocks	AA0023AL	http://www.aablocks.com/goods/Goods/detail?id=AA0023AL
AEchem Scientific Corp., USA	AE1-008053	http://www.aechemsc.com/info_products/AE1-008053.php
AHH Chemical co.,ltd	MT-21047	http://www.ahhchemical.com/product/MT-21047.html
AK Scientific, Inc. (AKSCI)	H331	http://www.aksci.com/item_detail.php?cat=H331
AK Scientific, Inc. (AKSCI)	HTS001426	http://www.aksci.com/item_detail.php?cat=HTS001426
Aaron Chemicals	AR00242D	http://www.aaronchem.com/1821-12-1
AbMole Bioscience	M9638	http://www.abmole.com/products/4-phenylbutyric-acid.html
Acadechem	ACDS-017220	http://www.acadechemical.com/product/91919
Accela ChemBio Inc	SY004771	http://www.accelachem.com/cn/search.html?keyword=SY004771&attname=GoodsCode
Achemica	ACMC-209eip	http://www.achemica.com/prodinfo/?id=124256
Achemo Scientific Limited	AC-38854	http://www.achemo.com/search/?Keyword=1821-12-1
Achemtek	0104-017382	http://www.achemtek.com/1821-12-1
Acorn PharmaTech	ACN-028755	http://www.acornpharmatech.com/
Acros Organics	AC130385000	https://www.fishersci.com/shop/products/4-phenylbutyric-acid-99-acros-organics-3/AC130385000
Acros Organics	AC130380250	https://www.fishersci.com/shop/products/4-phenylbutyric-acid-99-acros-organics/AC130380250
Acros Organics	AC130381000	https://www.fishersci.com/shop/products/4-phenylbutyric-acid-99-acros-organics/AC130381000
Alfa Aesar	A18115	https://www.alfa.com/en/catalog/A18115/
Alfa Chemistry	AP1716127	https://reagents.alfa-chemistry.com/product/sodium-phenylbutyrate-cas-1716-12-7-6398.html
Alichem	19115942	http://www.alichem.com/en/products/1821-12-1.html
Allbio Pharm Co., Ltd	AD08862	http://www.allbiopharm.com/product/n/AD08862
Ambinter	Amb2388283	http://www.ambinter.com/reference/2388283
Angene	AGN-PC-006I8K	http://www.angenechemical.com/productshow/AGN-PC-006I8K.html
Anward	ANW-23087	http://www.anward.com/pro_result/16412
Ark Pharm	AK-96640	http://www.arkpharminc.com/products/1821-12-1.html
Aromalake	LS10686	http://www.aromalake.com/4-Phenylbutyric-acid-p863.html
AstaTech, Inc.	CX1106	http://www.astatechinc.com/CPSResult.php?CRNO=12623
Aurora Fine Chemicals LLC	A00.448.959	http://online.aurorafinechemicals.com/info?ID=A00.448.959
Aurora Fine Chemicals LLC	K00.800.508	http://online.aurorafinechemicals.com/info?ID=K00.800.508
Aurum Pharmatech LLC	Z-3857	http://www.Aurumpharmatech.com/Product/ProductDetails/Z_3857
Avantor Inc	BT140580-100G	https://us.vwr.com/store/product/16638478/4-phenylbutyric-acid-99
Avantor Inc	BT140580-25G	https://us.vwr.com/store/product/16638478/4-phenylbutyric-acid-99
Avantor Inc	BT140580-500G	https://us.vwr.com/store/product/16638478/4-phenylbutyric-acid-99
BLD Pharm	BD12033	http://www.bldpharm.com/products/1821-12-1.html
BioChemPartner	BCP10715	http://www.biochempartner.com/1821-12-1-BCP10715
BioSynth	Q-200507	https://www.biosynth.com/en/products/chemical-intermediates/basic-intermediates/products/Q-200507.html
Boerchem	BC678065	http://www.boerchem.com/?product_39680.html
CSNpharm	CSN23643	https://www.csnpharm.com/products/4-phenylbutanoic-acid.html
Chem Scene	CS-D1686	http://www.chemscene.com/1821-12-1.html
ChemMol	30109593	http://www.chemmol.com/chemmol/30109593.html
ChemMol	44010138	http://www.chemmol.com/chemmol/44010138.html
ChemMol	49407152	http://www.chemmol.com/chemmol/49407152.html
ChemMol	96707547	http://www.chemmol.com/chemmol/96707547.html
ChemMol	97800148	http://www.chemmol.com/chemmol/97800148.html
Chemspace	CSSB00011207963	https://chem-space.com/CSSB00011207963
Clearsynth	CS-O-10122	https://www.clearsynth.com/en/CSO10122.html
DC Chemicals	DC37233	http://www.dcchemicals.com//product_show-4-Phenylbutyric_acid.html
Finetech	FT-0619401	http://www.finetechnology-ind.com/prod/detail/pub/1821-12-1.html
Finetech	FT-0673736	http://www.finetechnology-ind.com/prod/detail/pub/358730-86-6.html
Frinton	FR-2080	https://www.frinton.com/search/?filtername=4-Phenylbutyric Acid
Glentham Life Sciences	GK5010	http://www.glentham.com/products/product/GK5010/
Hairui	HR124591	http://www.hairuichem.com/en/product/1821-12-1.html
Heterocyclics Research Chemicals & Building blocks	JR-12561	http://www.heterocyclics.com/details.php?cat_id=JR-12561
Lab Network	LN00196776	https://labnetwork.com/frontend-app/p/#!/moleculedetails/LN00196776
Matrix Scientific	127423	https://www.matrixscientific.com/127423.html
MedChem Express	HY-A0281	https://www.medchemexpress.com/Benzenebutyric_acid.html
Molcore	MC533818	https://www.molcore.com/product/1821-12-1
Nextpeptide	NP184273	http://www.nextpeptide.com/1821-12-1.html
Oakwood	214820	http://www.oakwoodchemical.com/ProductsList.aspx?CategoryID=-2&txtSearch=95819&ExtHyperLink=1
Parchem	29860	https://www.parchem.com/chemical-supplier-distributor/4-Phenylbutyric-Acid-029860.aspx
R&D Chemicals	FR-2080	http://www.rdchemicals.com/chemicals.php?mode=details&mol_id=2996
Renno Tech	RP22720	http://www.rennotech.com/product_show.asp?id=28410
Selleck Chemicals	S3592	http://www.selleckchem.com/products/4-phenylbutyric-acid-4-pba-.html
Sigma-Aldrich	P21005_ALDRICH	https://www.sigmaaldrich.com/catalog/product/aldrich/p21005?utm_source=pubchem&utm_campaign=pubchem_2017&utm_medium=referral
Sinfoo Biotech	S001563	http://www.sinfoobiotech.com/product/1004961
Smolecule	S516219	http://www.smolecule.com/products/s516219
TCI (Tokyo Chemical Industry)	P0643	http://www.tcichemicals.com/eshop/en/us/commodity/P0643/index.html
THE BioTek	bt-256833	https://www.thebiotek.com/product/inhibitors/bt-256833
VladaChem	VL143786	https://www.vladachem.com/product.php?products=1821-12-1
VladaChem	VL266856-100G	https://www.vladachem.com/product.php?products=1821-12-1
Yuhao Chemical	RT12403	http://www.chemyuhao.com/1821-12-1.html
abcr GmbH	AB118474	http://www.abcr.com/shop/en/AB118474
eNovation Chemicals	D688024	https://www.enovationchem.com/ProductDetails.asp?ProductID=D688024
iChemical	EBD2199138	http://www.ichemical.com/products/1821-12-1.html?utm_source=PubChem&utm_medium=website&utm_campaign=product%20catalogs
mcule	MCULE-6322732346	https://mcule.com/MCULE-6322732346/

PubMed

Title	Date	PubMed
Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis.	2010 Jun	20512995
Sodium phenylbutyrate reverses lysosomal dysfunction and decreases amyloid-β42 in an in vitro-model of inclusion-body myositis.	2014 May	24472798
	2017 Apr	29381293
Safety and efficacy of glycerol phenylbutyrate for management of urea cycle disorders in patients aged 2months to 2years.	2017 Nov	28916119

Patents

Sample Use Guides

In Vivo Use Guide

Tablets is indicated for children weighing more than 20 kg and for adults. The usual total daily dose of Tablets and Powder for patients with urea cycle disorders is 450–600 mg/kg/day in patients weighing less than 20 kg, or 9.9–13.0 g/m2/day in larger patients. The tablets and powder are to be taken in equally divided amounts with each meal or feeding (i.e., three to six times per day). Powder is indicated for oral use (via mouth, gastrostomy, or nasogastric tube) only.

Route of Administration: Oral

In Vitro Use Guide

Unknown

Name

Type

Language

GLYCEROL PHENYLBUTYRATE

Official Name

English

GLYCEROL PHENYLBUTYRATE [VANDF]

Common Name

English

GLYCEROL PHENYLBUTYRATE [WHO-DD]

Common Name

English

GLYCEROL PHENYLBUTYRATE [USAN]

Common Name

English

GLYCEROL PHENYLBUTYRATE [MI]

Common Name

English

HPN-100

Code

English

TRI(4-PHENYLBUTYRYL)GLYCEROL

Systematic Name

English

glycerol phenylbutyrate [INN]

Common Name

English

BENZENEBUTANOIC ACID, 1,2,3-PROPANETRIYL ESTER

Common Name

English

GLYCEROL PHENYLBUTYRATE [ORANGE BOOK]

Common Name

English

GLYCERYL TRI (4-PHENYLBUTYRATE)

Systematic Name

English

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

Systematic Name

English

HPN100

Code

English

RAVICTI

Brand Name

English

GT4P

Common Name

English

BENZENEBUTANOIC ACID, 1,1',1''-(1,2,3-PROPANETRIYL) ESTER

Common Name

English

GLYCERYL TRI(4-PHENYLBUTYRATE)

Systematic Name

English

GLYCERYL TRI-(4-PHENYLBUTYRATE)

Systematic Name

English

Classification Tree Code System Code

FDA ORPHAN DRUG

204205