Carnitine synthesis

Stable Identifier
Homo sapiens
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Carnitine is required for the shuttling of fatty acids into the mitochondrial matrix and its deficiency is associated with metabolic diseases. It is abundant in a typical Western diet but can also be 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 & Hoppel 1998; Rebouche & Engel 1980; Vaz & Wanders 2002).
Literature References
PubMed ID Title Journal Year
6770910 Tissue distribution of carnitine biosynthetic enzymes in man

Engel, AG, Rebouche, CJ

Biochim Biophys Acta 1980
6361812 Carnitine--metabolism and functions

Bremer, J

Physiol. Rev. 1983
11802770 Carnitine biosynthesis in mammals

Vaz, FM, Wanders, RJA

Biochem J 2002
9706223 Genetic disorders of carnitine metabolism and their nutritional management.

Hoppel, C, Kerner, J

Annu Rev Nutr 1998
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