Sodium phenylbutyrate is a prodrug, it rapidly metabolized into acid salt. It can be combined with glutamic acid and ammonia generated phenylacetyl glutamine, through renal excretion, excretion of nitrogenous waste is another way.
Sodium Phenylbutyrate is a low molecular weight fatty acid that has been used for treatment of urea cycle disorders in children, sickle cell disease, and thalassemia.
Sodium Phenylbutyrate has been demonstrated recently that 4-PBA can act as a chemical chaperone by reducing the load of mutant or mislocated proteins retained in the endoplasmic reticulum (ER) under conditions associated with cystic fibrosis and liver injury.
CAS: 1716-12-7
Molecular Formula: C10H11O2.Na
Molecular Weight: 163.20+22.99 g/mol
