Glycerol Phenylbutyrate Suppliers & Bulk Manufacturers

Available Forms: Oral Solution

Available Strengths: 1.1 g/mL

Reference Brands: Ravicti (USA)

Category: Orphan Drugs

Product Description:

Glycerol phenylbutyrate is a nitrogen-binding agent used for the chronic management of urea cycle disorders and is marketed under the brand name Ravicti. It is indicated for patients who cannot be adequately managed with dietary protein restriction and/or amino acid supplementation alone. Urea cycle disorders are rare inherited metabolic conditions that result in the accumulation of ammonia in the blood, which can be toxic to the brain and other organs.

Glycerol phenylbutyrate works by providing an alternative pathway for the elimination of excess nitrogen from the body. After oral administration, it is metabolized into phenylbutyric acid, which is further converted to phenylacetate. Phenylacetate conjugates with glutamine to form phenylacetylglutamine, a compound that is excreted through the kidneys. This process effectively removes nitrogen and helps reduce elevated ammonia levels in the bloodstream.

Designed for long-term use, glycerol phenylbutyrate allows for continuous control of hyperammonemia in both adult and pediatric patients. Its liquid oral formulation supports accurate dosing and improved tolerability, making it suitable for chronic therapy. Glycerol phenylbutyrate plays a critical role in the ongoing management of urea cycle disorders under specialist medical supervision.