1.14.11.8 additional information enzyme is localized to the mitochondrial matrix, product formation is limited by 6-N-trimethyllysine transport across the mitochondrial inner membrane 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 carnitine biosynthesis 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 first enzyme of the L-carnitine biosynthesis