Carnitine biosynthesis
Carnitine biosynthesis is a method for the endogenous production of L-carnitine, a molecule that is essential for energy metabolism. In humans and many other animals, L-carnitine is obtained from both diet and by biosynthesis. The carnitine biosynthesis pathway is highly conserved among many eukaryotes and some prokaryotes.
L-Carnitine is biosynthesized from Nε-trimethyllysine. At least four enzymes are involved in the overall biosynthetic pathway. They are Nε-trimethyllysine hydroxylase, 3-hydroxy-Nε-trimethyllysine aldolase, 4-N-trimethylaminobutyraldehyde dehydrogenase and γ-butyrobetaine hydroxylase.
The first enzyme of the L-carnitine biosynthetic pathway is Nε-trimethyllysine hydroxylase. Nε-trimethyllysine hydroxylase is an iron(II) and 2-oxoglutarate (2OG) dependent oxygenase.Nε-trimethyllysine hydroxylase catalyses the hydroxylation reaction of Nε-trimethyllysine to 3-hydroxy-Nε-trimethyllysine.
...
Wikipedia