PEDIATRICS Vol. 40 No. 6 December 1967, pp. 986-992
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PATHS OF LYSINE DEGRADATION IN PATIENTS WITH HYPERLYSINEMIA

Norman C. Woody M.D.1 and Eng Bee Ong Ph.D.1

1 Departments of Pediatrics and Biochemistry, Tulane Medical School, and the Charity Hospital of Louisiana, New Orleans

Study of lysine metabolites in the urine of two children with hyperlysinemia indicates that several pathways for lysine breakdown exist in man. Two of these are demonstrated to be functioning and are shown to be of secondary importance for lysine degradation in these children. One of the paths, via homocitrulline and homoarginine, has been demonstrated for humans by others. The second, through \g=elunate\-N-acetyl-L-lysine, is documented here for the first 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.

Submitted on March 6, 1967
Accepted on July 12, 1967