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 Rattus norvegicus ? - ? 89 1.14.11.8 additional information determination of the (2S,3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine and N6,N6,N6-trimethyl-L-lysine is performed by ultraperformance liquid chromatography–tandem mass spectrometry (UPLC/MS/MS) in positive ion electrospray mode Homo sapiens ? - ? 89 1.14.11.8 additional information NMR spectrosopic structure analysis of substrates and products Homo sapiens ? - ? 89 1.14.11.8 additional information NMR spectrosopic structure analysis of substrates and products, substrate specificity, overview. No or poor activity with dimethyllysine, methyllysine, lysine, D-trimethyllysine, 5-trimethylamino-1-aminopentane, trimethyllysine, 6-trimethylaminohexanoic acid, hexamethyllysine, N-acetyltrimethyllysine, trimethyldiaminobutyric acid, triethyllysine, symmetric and asymmetric dimethylarginines, mildronate, and gamma-butyrobetaine, structure-activity relationship study Homo sapiens ? - ? 89 1.14.11.8 N5,N5,N5-trimethyl-L-ornithine + 2-oxoglutarate + O2 - Homo sapiens (3S)-3-hydroxy-N5,N5,N5-trimethyl-L-ornithine + succinate + CO2 - ? 443777 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - Mus musculus 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - Homo sapiens 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - Rattus norvegicus 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - Sus scrofa 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - Neurospora crassa 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 N6,N6, N6-trimethyl-L-lysine is epsilon-N-trimethyl-L-lysine Rattus norvegicus 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 N6,N6, N6-trimethyl-L-lysine is epsilon-N-trimethyl-L-lysine Bos taurus 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 N6,N6, N6-trimethyl-L-lysine is epsilon-N-trimethyl-L-lysine Neurospora crassa 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 carnitine biosynthesis Rattus norvegicus 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 first enzyme of the L-carnitine biosynthesis Rattus norvegicus 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 2718 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 - Homo sapiens (3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 442485 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 the enzymatic hydroxylation occurs at the C-3 site of (2S)-Nepsilon-trimethyllysine substrate. Comparative NMR spectroscopic studies (with two enantiopure synthetic standards that possess 3R and 3S stereochemistry) on the enzymatically produced (2S)-3-hydroxy-Nepsilon-trimethyllysine reveal that TMLH exclusively catalyzes the formation of (3S)-3-hydroxy-Nepsilon-trimethyl-L-lysine, not forming any (3R)-3-hydroxy-Nepsilon-trimethyl-L-lysine diastereoisomer Homo sapiens (3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 442485 1.14.11.8 N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 recognition sites that contribute to the enzymatic activity of the enzyme (TMLH): the Fe(II)-binding H242-D244-H389 residues, R391-R398 involved in 2-oxoglutarate-binding, and several residues (D231, N334, and the aromatic cage comprised of W221, Y217 and Y234) associated with binding of (2S)-Nepsilon-trimethyllysine Homo sapiens (3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 - ? 442485 1.14.11.8 N6,N6-dimethyl-N6-ethyl-L-lysine + 2-oxoglutarate + O2 - Homo sapiens (3S)-3-hydroxy-N6,N6-dimethyl-N6-ethyl-L-lysine + succinate + CO2 - ? 443778 1.14.11.8 N6,N6-dimethyl-N6-isopropyl-L-lysine + 2-oxoglutarate + O2 - Homo sapiens (3S)-3-hydroxy-N6,N6-dimethyl-N6-isopropyl-L-lysine + succinate + CO2 - ? 443779 1.14.11.8 N6,N6-dimethyl-N6-propyl-L-lysine + 2-oxoglutarate + O2 - Homo sapiens (3S)-3-hydroxy-N6,N6-dimethyl-N6-propyl-L-lysine + succinate + CO2 - ? 443780 1.14.11.8 N6-fluoromethyl-N6,N6-dimethyl-L-lysine + 2-oxoglutarate + O2 Nepsilon-trimethyllysine analogue that contains the fluoromethyl group can be used as a 1H and 19F NMR probe for studies on TMLH catalysis Homo sapiens (3S)-3-hydroxy-N6-fluoromethyl-N6,N6-dimethyl-L-lysine + succinate + CO2 - ? 443781 1.14.11.8 N7,N7,N7-trimethyl-L-alpha-homolysine + 2-oxoglutarate + O2 - Homo sapiens (3S)-3-hydroxy-N7,N7,N7-trimethyl-L-alpha-homolysine + succinate + CO2 - ? 443782