is essential to the synthesis of carnitine.
Inhibition of carnitine biosynthesis by valproic acid in rats—The ..
Carnitine biosynthesis. Several enzymatic reactions are needed for ‐carnitine synthesis. (1) Lysyl methyltransferases, (2) trimethyllysine dioxygenase, (3) hydroxyl ‐trimethyllysine aldolase, (4) 4‐trimethylammoniobutyraldehyde dehydrogenase and (5) γ‐butyrobetaine hydroxylase.
Enzymology of the carnitine biosynthesis pathway
N2 - Liver and testis slices convert 6 N trimethyl lysine into 4 N trimethylaminobutyrate and carnitine. Adipose, skeletal muscle, heart, or kidney tissues metabolize trimethyl lysine into trimethylaminobutyrate but not into carnitine. Trimethylaminobutyrate hydroxylation, forming carnitine, occurs in liver and to a minor degree in testis. Liver is the primary site of carnitine biosynthesis in the rat.