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This gene encodes the protein trimethyllysine dioxygenase which is the first enzyme in the carnitine biosynthesis pathway. Carnitine play an essential role in the transport of activated fatty acids across the inner mitochondrial membrane. The encoded protein converts trimethyllysine into hydroxytrimethyllysine. A pseudogene of this gene is found on chromosome X. Alternate splicing results in multiple transcript variants.[provided by RefSeq, May 2010]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0005506 [iron ion binding] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0009083 [branched-chain amino acid catabolic process] GO:0016491 [oxidoreductase activity] GO:0016702 [oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen] GO:0031418 [L-ascorbic acid binding] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0045329 [carnitine biosynthetic process] GO:0050353 [trimethyllysine dioxygenase activity] GO:0051354 [negative regulation of oxidoreductase activity] GO:0055114 [oxidation-reduction process]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)