Carnitine deficiency in lysinuric protein intolerance: lysine-sparing effect of carnitine.

Abstract

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 less than 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.

• Authors: G Takada, A Goto, K Komatsu, R Goto
• Journal: The Tohoku journal of experimental medicine (Tohoku J Exp Med) Vol. 153 Issue 4 Pg. 331-4 (Dec 1987) ISSN: 0040-8727 [Print] Japan
• PMID: 3126568 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Lysine
• Carnitine

Topics

• Amino Acid Metabolism, Inborn Errors (diet therapy, metabolism)
• Carnitine (administration & dosage, blood, deficiency)
• Child
• Humans
• Lysine (blood, deficiency, urine)
• Male