Rx00218 - Sodium Phenylbutyrate


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Rx00218
Primary Indication
Secondary Indication

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Disease prevalence from Electronic Health Records

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Disease A Disease B A∩B A∩¬B B∩¬A Odds-Ratio P-Value Adjusted P-Value
hyperammonemic encephalopathy deficiency of ornithine transcarbamylase 743 743 743 5430.584 0 0.0
deficiency of carbamylphosphate synthetase deficiency of ornithine transcarbamylase 743 743 743 5430.584 0 0.0
deficiency of argininosuccinic acid synthetase deficiency of ornithine transcarbamylase 743 743 743 5430.584 0 0.0
deficiency of ornithine transcarbamylase urea cycle disorders 743 743 743 5430.584 0 0.0
urea cycle disorders deficiency of ornithine transcarbamylase 743 743 743 5430.584 0 0.0

Chemical, Pharmacological and Biological Annotations


ATC Code:
A16AX09
Category:
Ammonia Detoxicants
KEGG Drug ID:
D10127
SMILE:
O=C(CCCC1=CC=CC=C1)OCC(COC(=O)CCCC1=CC=CC=C1)OC(=O)CCCC1=CC=CC=C1
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
Classification:
Kingdom:
Organic Compounds
Superclass:
Lipids
Class:
Glycerolipids
Subclass:
Triacylglycerols
Indication:
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 supplemen...
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Pharmacodynamics:
Glycerol phenylbutyrate prolongs the QTc interval.
Description:
Glycerol phenylbutyrate is a nitrogen-binding agent. Chemically, it is a triglyceride in which three molecules of phenylbutyrate are linked to a glycerol backbone. FDA approved on February 1, 2013.
Mechanism:
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...
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Adsorption, Distribution, Metabolism, Excretion and Toxicity


Human Intestinal Absorption:
+
Blood Brain Barrier:
+
Caco-2 permeable:
+
P-glycoprotein substrate:
Non-substrate
P-glycoprotein inhibitor I:
Inhibitor
P-glycoprotein inhibitor II:
Inhibitor
Renal organic cation transporter:
Non-inhibitor
CYP450 2C9 substrate:
Inhibitor
CYP450 2D6 substrate:
Non-inhibitor
CYP450 2C19 substrate:
Inhibitor
CYP450 inhibitory promiscuity:
High CYP Inhibitory Promiscuity
Ames test:
Non AMES toxic
Carcinogenicity:
Non-carcinogens
Biodegradation:
Ready biodegradable
Rat acute toxicity:
1.7748 LD50, mol/kg
hERG inhibition (predictor I):
Weak inhibitor
hERG inhibition (predictor II):
Non-inhibitor

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Dudley Laboratory
Department of Genetics and Genomic Sciences
Icahn Institute for Genomics and Multiscale Biology
Icahn School of Medicine at Mount Sinai
One Gustave L. Levy Place, Box 1498
Manhattan, New York City, NY
repurposedb@dudleylab.org