Save up -80% on Glycerol Phenylbutyrate
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2018 Price of Ravicti
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Glycerol Phenylbutyrate volume of distribution
Discount Cards 16,000+
Clients Benefit 29%
Total savings $4,735,080
Glycerol Phenylbutyrate mechanism of action
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.
Dosage forms of Glycerol Phenylbutyrate
Glyceryl Tri-4-Phenylbutyrate GPB
Horizon Therapeutics, Llc.
Humans and other mammals
Indication of Glycerol Phenylbutyrate
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.
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