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MGP003548

UniProt Annotations

Entry Information
Gene Namebutyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1
Protein EntryBODG_HUMAN
UniProt IDO75936
SpeciesHuman
Comments
Comment typeDescription
Catalytic Activity4-trimethylammoniobutanoate + 2-oxoglutarate + O(2) = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO(2). {ECO:0000269|PubMed:20599753}.
CofactorName=Fe(2+); Xref=ChEBI:CHEBI:29033; Evidence={ECO:0000305}; Note=Binds 1 Fe(2+) ion per subunit. {ECO:0000305};
CofactorName=L-ascorbate; Xref=ChEBI:CHEBI:38290;
FunctionCatalyzes the formation of L-carnitine from gamma- butyrobetaine.
PathwayAmine and polyamine biosynthesis; carnitine biosynthesis.
SimilarityBelongs to the gamma-BBH/TMLD family. {ECO:0000305}.
Subcellular LocationCytoplasm.
Tissue SpecificityHighly expressed in kidney; moderately expressed in liver; very low expression in brain.
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