Phenylbutyrate is used in urea cycle disorders because it
In all urea cycle disorders there is accumulation of NH3 (hyperammonemia) which is very toxic and has to be removed Using scavenging agents:- One of the treatment of urea cycle disorder is the use of NH3 scavenging agents These are Phenylbutyrate and Sodium benzoate Phenyl butyrate reacts with glutamine to get conveed in to phenyl acetyl glutamine which can be excreted. Other method to treat urea cycle disorders: Give Arginine (1st line Treatment ) An essential amino acid Provides ornithine (required in 2nd step of urea cycle) Activator of NAGS But it is contraindicated in Arginase deficiency Restrict protein intake to 50% of normal
Scavenges nitrogen
Activates enzymes
Maintains renal output
Maintains energy production
Urea cycle