About: Glycerol Phenylbutyrate

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About: Glycerol Phenylbutyrate Goto Sponge NotDistinct Permalink

An Entity of Type : http://linked.opendata.cz/ontology/drugbank/Drug, within Data Space : linked.opendata.cz associated with source document(s)

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rdf:type	http://linked.opendata.cz/ontology/drugbank/Drug
http://linked.open...gbank/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. (en)
http://linked.open...y/drugbank/dosage	http://linked.opendata.cz/resource/drugbank/dosage/271B5C56-363D-11E5-9242-09173F13E4C5
http://linked.open...generalReferences	# FDA label (en)
http://linked.open...gy/drugbank/group	approved (en)
http://linked.open...ugbank/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 supplementation alone. (en)
sameAs	Glycerol Phenylbutyrate
Title	Glycerol Phenylbutyrate (en)
adms:identifier	http://linked.opendata.cz/resource/drugbank/drug/DB08909/identifier/drugbank/DB08909 http://linked.opendata.cz/resource/drugbank/drug/DB08909/identifier/kegg-drug/D10127 http://linked.opendata.cz/resource/drugbank/drug/DB08909/identifier/national-drug-code-directory/76325-100-25 http://linked.opendata.cz/resource/drugbank/drug/DB08909/identifier/wikipedia/Glycerol_phenylbutyrate
http://linked.open...mechanismOfAction	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. (en)
http://linked.open...outeOfElimination	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. (en)
http://linked.open.../drugbank/synonym	GPB (en) Glyceryl Tri-4-Phenylbutyrate (en) HPN100 (en) Ravicti (en)
http://linked.open...drugbank/toxicity	Most common adverse reactions in ≥10% of patients are diarrhea, flatulence, and headache. (en)
http://linked.open.../drug/hasAHFSCode	http://linked.opendata.cz/resource/AHFS/40-10
http://linked.open...nk/proteinBinding	PBA = 80.6% to 98.0%; PAA = 37.1% to 65.6%; PAGN = 7% to 12%. (en)
foaf:page	http://www.drugs.com/cdi/glycerol-phenylbutyrate.html http://www.rxlist.com/ravicti-drug.htm
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http://linked.open...l/drug/hasATCCode	Glycerol phenylbutyrate
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http://linked.open...ugbank/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 µg/mL; PAA = 14.9 µg/mL; PAGN = 30.2 µg/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. (en)
http://linked.open.../affectedOrganism	Humans and other mammals (en)
http://linked.open...casRegistryNumber	611168-24-2 (en)
http://linked.open...drugbank/category	(en)
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