Metabolism and Bioavailability
In healthy people, carnitine homeostasis (balance) is maintained through endogenous biosynthesis of L-carnitine, absorption of carnitine from dietary sources, and elimination and reabsorption of carnitine by the kidneys (5).
Endogenous biosynthesis
Humans can synthesize L-carnitine from the amino acids lysine and methionine in a multi-step process. Specifically, protein-bound lysine is enzymatically methylated to form episilon-N-trimethyllysine; three molecules of methionine provide the methyl groups for the reaction. Epsilon-N-trimethyllysine is released for carnitine synthesis by protein hydrolysis (5, 6). Several enzymes are involved in endogenous L-carnitine biosynthesis. The enzyme γ-butyrobetaine hydroxylase, however, is absent from cardiac and skeletal muscle but highly expressed in human liver, testes, and kidney (7). The rate of L-carnitine biosynthesis in humans was studied in vegetarians and is estimated to be 1.2 micromol/kg of body weight/day (8). Changes in dietary carnitine intake or renal reabsorption do not appear to affect the rate of endogenous carnitine synthesis