Carnitine biosynthesis
From Wikipedia, the free encyclopedia
Carnitine biosynthesis is a method for the endogenous production of L-carnitine, a molecule that is essential for energy metabolism.[1][2][3][4] In humans and many other animals, L-carnitine is obtained from both diet and by biosynthesis.[5][6] The carnitine biosynthesis pathway is highly conserved among many eukaryotes and some prokaryotes.[7][8][9]
L-Carnitine is biosynthesized from Nε-trimethyllysine.[10] 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.