1.14.11.8 evolution the enzyme belongs to the family of 2-oxoglutarate (2OG)-dependent oxygenases 744681 1.14.11.8 evolution the enzyme belongs to the family of 2-oxoglutarate (2OG)-dependent oxygenases. Members of Fe(II) and 2-oxoglutarate-dependent oxygenases catalyze stereoselective hydroxylations of unactivated C-H bonds in various (bio)molecules, including proteins, DNA, and small molecule metabolites 745961 1.14.11.8 evolution the enzyme belongs to the non-heme Fe(II) and 2-oxoglutarate dependent oxygenases 744678 1.14.11.8 metabolism the enzyme catalyses the first step in mammalian biosynthesis of carnitine, which plays a crucial role in fatty acid metabolism. Carnitine biosynthesis patway, overview 744681 1.14.11.8 metabolism the enzyme catalyzes the first step in the biosynthesis of carnitine 691166 1.14.11.8 metabolism the enzyme catalyzes the first step in the biosynthesis of carnitine, i.e. L-3-hydroxy-4-N-N-N-trimethylaminobutyrate. Lysine in protein peptide linkages undergoes methylation of the 1-amino group to yield trimethyllysine, TML, which is released upon protein degradation. Muscle is the major source of TML. The released TML is further oxidised to gamma-butyrobetaine by the action of trimethyllysine dioxygenase, 3-hydroxy-N-trimethyllysine aldolase and 4-N-trimethylaminobutyraldehyde dehydrogenase 697027 1.14.11.8 metabolism trimethyllysine hydroxylase (TMLH) catalyses C-3 hydroxylation of Nepsilon-trimethyllysine in the first step of carnitine biosynthesis 744678 1.14.11.8 metabolism trimethyllysine hydroxylase (TMLH) catalyses C-3 hydroxylation of Nepsilon-trimethyllysine in the first step of carnitine biosynthesis in humans 745958 1.14.11.8 metabolism trimethyllysine hydroxylase (TMLH) catalyses the first step in carnitine biosynthesis, the conversion of N6,N6,N6-trimethyl-L-lysine to 3-hydroxy-N6,N6,N6-trimethyl-L-lysine 746366 1.14.11.8 metabolism trimethyllysine hydroxylase (TMLH) is involved in the first step of the physiologically important carnitine biosynthesis pathway. The enzyme catalyzes C-3 hydroxylation of (2S)-Nepsilon-L-trimethyllysine, to produce 3-hydroxy-Nepsilon-L-trimethyllysine, which then undergoes three additional enzymatic steps to the final L-carnitine 745961