Glycerol phenylbutyrate
≥99%
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Glycerol phenylbutyrate is primarily used in the treatment of urea cycle disorders (UCDs), which are genetic conditions that lead to the accumulation of ammonia in the blood. It works by providing an alternative pathway for nitrogen excretion, reducing the toxic buildup of ammonia. This medication is often prescribed for patients who cannot manage their condition through diet alone or who are not suitable candidates for other treatments like sodium phenylbutyrate. It is administered orally, making it convenient for long-term use, particularly in pediatric and adult patients. The compound is metabolized in the body to phenylacetate, which conjugates with glutamine to form phenylacetylglutamine, a substance excreted in the urine, thereby helping to lower ammonia levels. Its effectiveness and tolerability make it a critical therapeutic option for managing UCDs and improving patient quality of life.
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