1987 Volume 153 Issue 4 Pages 331-334
TAKADA, G., GOTO, A., KOMATSU, K. and GOTO, R. Carnitine Deficiency in Lysinuric Protein Intolerance: Lysine-Sparing Effect of Carnitine. Tohoku J. exp. Med., 1987, 153 (4), 331-334 - Plasma carnitine deficiency (41% of normal control) was found in a 9 year-old boy with lysinuric protein intolerance. Following oral carnitine administration, the plasma level of lysine as well as carnitine has significantly increased (p<0.05). His well-being seemed to have improved on this substitutive remedy. Hitherto unrevealed these findings on LPI indicate that carnitine has a lysine-sparing effect and suggest that there might be a biosynthetic pathway of carnitine directly from free lysine in human.
