Carnitine biosynthesis

Carnitine biosynthesis is a method for the endogenous production of L-carnitine, a molecule that is essential for energy metabolism.

The first enzyme of the L-carnitine biosynthetic pathway is Nε-trimethyllysine hydroxylase, an iron and 2-oxoglutarate (2OG)-dependent oxygenase that also requires ascorbate.

[citation needed] High-performance liquid chromatography (HPLC) analysis has confirmed that vegetables contain a significant amount of Nε-trimethyllysine.

The final step of L-carnitine biosynthesis is γ-butyrobetaine hydroxylase, a zinc binding enzyme.

[36] Recombinant human γ-butyrobetaine hydroxylase has also been produced by Escherichia coli[27] and baculoviruses[26] systems.