L-Homocitrulline is an amino acid and can be detected in larger amounts in the urine of individuals with urea cycle disorders. At present, it is thought...

phosphate + L-lysine ⇌ {\displaystyle \rightleftharpoons } phosphate + L-homocitrulline Thus, the two substrates of this enzyme are carbamoyl phosphate and...

will show elevated ornithine levels, and urine amino acids will detect homocitrulline. Orotic acid may also be elevated. Ammonia levels can be variably elevated...

doi:10.1016/S0021-9258(19)45215-0. Ryan, W. L.; Wells, I. C. (1964). "Homocitrulline and Homoarginine Synthesis from Lysine". Science. 144 (3622): 1122–7...

particularly in neonatal onset cases to identify the presence or absence of homocitrulline for ruling out ornithine translocase deficiency (hyperornithinemia,...