Abstract
In humans, L-carnitine (L- (-) -3-hydroxy-4-trimethylaminobutyrate, molecular weight 62 daltons) is synthesized in the liver, kidney and brain from the two essential amino acids lysine and methionine mediated by a multi-enzyme system1. Four co-factors — vitamin C, niacin, vitamin B6, and iron — are required by the various enzymes2 , 3 . The rare amino acid N-6-trimethyllysine (TML) is formed by posttranslational methylation of lysine residues with S-adenosylmethionine4. Once released by proteolytic action, it may serve as a precursor for the endogenous biosynthesis of carnitine5 . TML is converted to L-carnitine via four enzymatic steps (hydroxylation, aldol cleavage, oxidation and a second hydroxylation).
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© 1989 Plenum Press, New York
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Fürst, P., Glöggler, A., Rössle, C. (1989). Carnitine Supplementation in Uremia. In: Hörl, W.H., Schollmeyer, P.J. (eds) New Perspectives in Hemodialysis, Peritoneal Dialysis, Arteriovenous Hemofiltration, and Plasmapheresis. Advances in Experimental Medicine and Biology, vol 260. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-5718-6_8
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DOI: https://doi.org/10.1007/978-1-4684-5718-6_8
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