L-Homocitrulline

Description: L-Homocitrulline (Homocitrulline) is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders. The accumulation of carbamyl phosphate due to a depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of L-Homocitrulline and homoarginine in some cases. Target: Others, Endogenous Metabolite. Smiles: NC(CCCCNC(N)=O)C(O)=O. References: Kato T, et al. Homocitrullinuria and homoargininuria in lysinuric protein intolerance. J Inherit Metab Dis. 1989,12(2):157-61.