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This gene encodes gamma butyrobetaine hydroxylase which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. [provided by RefSeq, Jul 2008]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Protein evidence (Ezkurdia et al 2014)
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GO:0005506 [iron ion binding] GO:0005515 [protein binding] GO:0005829 [cytosol] GO:0008270 [zinc ion binding] GO:0008336 [gamma-butyrobetaine dioxygenase activity] GO:0009083 [branched-chain amino acid catabolic process] GO:0016491 [oxidoreductase activity] GO:0016706 [oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0045329 [carnitine biosynthetic process] GO:0055114 [oxidation-reduction process] GO:0070062 [extracellular exosome]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0005506 [iron ion binding] GO:0005515 [protein binding] GO:0005829 [cytosol] GO:0008270 [zinc ion binding] GO:0008336 [gamma-butyrobetaine dioxygenase activity] GO:0009083 [branched-chain amino acid catabolic process] GO:0016491 [oxidoreductase activity] GO:0016706 [oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0045329 [carnitine biosynthetic process] GO:0055114 [oxidation-reduction process] GO:0070062 [extracellular exosome]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0005506 [iron ion binding] GO:0005515 [protein binding] GO:0005829 [cytosol] GO:0008270 [zinc ion binding] GO:0008336 [gamma-butyrobetaine dioxygenase activity] GO:0009083 [branched-chain amino acid catabolic process] GO:0016491 [oxidoreductase activity] GO:0016706 [oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0045329 [carnitine biosynthetic process] GO:0055114 [oxidation-reduction process] GO:0070062 [extracellular exosome]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0005506 [iron ion binding] GO:0005515 [protein binding] GO:0005739 [mitochondrion] GO:0005829 [cytosol] GO:0008270 [zinc ion binding] GO:0008336 [gamma-butyrobetaine dioxygenase activity] GO:0009083 [branched-chain amino acid catabolic process] GO:0016491 [oxidoreductase activity] GO:0016706 [oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0045329 [carnitine biosynthetic process] GO:0055114 [oxidation-reduction process] GO:0070062 [extracellular exosome]