PATHS OF LYSINE DEGRADATION IN PATIENTS WITH HYPERLYSINEMIA

Abstract

Study of lysine metabolites in the urine fo 2 children with hyperlysinemia indicates that several pathways for lysine breakdown exist in man. Two of these were functioning and were shown to be of secondary importance for lysine degradation in these children. One of the paths, via homocitrulline and homoarginine, was demonstrated for humans by other studies. The second, through e-N-acetyl-L-lysine. is documented here for the 1st time in man. These pathways are not the site of the metabolic block, and another path of lysine degradation must be postulated as the site of the metabolic block in these patients with hyperlysinemia.