Essential knowledge of carnitine and carnitine deficiency
Journal Title: Journal of Analytical Bio-Science - Year 2012, Vol 35, Issue 4
Abstract
Carnitine is produced endogenously in the kidneys and liver, and is enriched in mutton and beef. It plays essential roles in the transportation of long-chain fatty acids into the mitochondria for beta-oxidation, in the elimination of acyl residues by carnitine binding, and in the facilitation of the mitochondrial metabolism by maintaining a free to acylated CoA ratio. Primary carnitine deficiency is caused by some inborn errors of metabolism, while secondary carnitine deficiency is caused by the administration of valproic acid, formula milk for the treatment, pivalate-conjugated antibiotics, etc. In a carnitine deficiency, fatty liver, hypoglycemia, and hyperammonemia have been observed, particularly in infants. Since a Reye-like syndrome is sometimes fatal in infants, carnitine is very important.
Authors and Affiliations
Tomoyoshi Matsui, Toshiaki Watanabe
Keywords
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