Carnitine synthesis

Stable Identifier
Homo sapiens
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Carnitine is synthesized in four steps from trimethyllysine (generated in turn by the S-adenosyl-methionine-mediated methylation of lysine residues in proteins, followed by protein hydrolysis). The enzymes that catalyze the first three steps of carnitine synthesis, converting trimethyllysine to gamma-butyrobetaine, are widely distributed in human tissues. The enzyme that catalyzes the last reaction, converting gamma-butyrobetaine to carnitine, is found only in liver and kidney cells, and at very low levels in brain tissues. Other tissues that require carnitine, such as muscle, are dependent on transport systems that mediate its export from the liver and uptake by other tissues (Bremer 1983, Kerner and Hoppel 1998).

Literature References
PubMed ID Title Journal Year
9706223 Genetic disorders of carnitine metabolism and their nutritional management.

Kerner, J, Hoppel, C

Annu Rev Nutr 1998
6361812 Carnitine--metabolism and functions

Bremer, J

Physiol. Rev. 1983
Participant Of
Event Information
Orthologous Events
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