Phenylbutyrate is used in management of urea cycle disorders. What is its role?
(A) Activates enzymes of urea cycle (B) Excretion of products of urea cycle (C) Maintains energy production (D) Scavenges nitrogen
(D) Answer- D. Scavenges nitrogenPhenylbutyrate is used to treat urea cycle disorders, because its metabolites offer an alternative pathway to the urea cycle to allow excretion of excess nitrogen. Urea cycle disorders result in the accumulation of precursors of urea, principally ammonia and glutamine. Phenylbutyrate provides an alternate means of detoxfication of glutamine acetylation, which bypasses the urea cycle.