Phenylbutyric acid
- Generic Name
- Phenylbutyric acid
- Brand Names
- Ammonaps, Buphenyl, Olpruva 2 Gm Pack, Pheburane, Relyvrio
- Drug Type
- Small Molecule
- Chemical Formula
- C10H12O2
- CAS Number
- 1821-12-1
- Unique Ingredient Identifier
- 7WY7YBI87E
- Background
Phenylbutyric acid is a fatty acid and a derivative of butyric acid naturally produced by colonic bacteria fermentation. It demonstrates a number of cellular and biological effects, such as relieving inflammation and acting as a chemical chaperone. It is used to treat genetic metabolic syndromes, neuropathies, and urea cycle disorders.
- Indication
Phenylbutyric acid is used for the treatment of various conditions, including urea cycle
disorders, neonatal-onset deficiency, late-onset deficiency disease in patients with a history of hyperammonemic encephalopathy. Phenylbutyric acid must be combined with dietary protein restriction and, in some cases, essential amino acid supplementation.
Phenylbutyric acid, as sodium phenylbutyrate, is used in combination with tauroursodeoxycholic acid to treat amyotrophic lateral sclerosis (ALS) in adults.
- Associated Conditions
- Amyotrophic Lateral Sclerosis (ALS), Argininosuccinate Synthetase Deficiency (Citrullinemia), Carbamyl Phosphate Synthetase Deficiency, Ornithine Transcarbamylase Deficiency, Deficiencies in enzymes of the urea cycle, Neonatal-onset deficiency
- Associated Therapies
- -
AMGEN REPORTS THIRD QUARTER 2024 FINANCIAL RESULTS
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