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MT000656

UniProt Annotations

Entry Information

Gene Name	butyrobetaine (gamma), 2-oxoglutarate dioxygenase (gamma-butyrobetaine hydroxylase) 1
Protein Entry	BODG_HUMAN
UniProt ID	O75936
Species	Human

Comments

Comment type	Description
Catalytic Activity	4-trimethylammoniobutanoate + 2-oxoglutarate + O(2) = 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO(2). {ECO:0000269\|PubMed:20599753}.
Cofactor	Name=Fe(2+); Xref=ChEBI:CHEBI:29033; Evidence={ECO:0000305}; Note=Binds 1 Fe(2+) ion per subunit. {ECO:0000305};
Cofactor	Name=L-ascorbate; Xref=ChEBI:CHEBI:38290;
Function	Catalyzes the formation of L-carnitine from gamma- butyrobetaine.
Pathway	Amine and polyamine biosynthesis; carnitine biosynthesis.
Similarity	Belongs to the gamma-BBH/TMLD family. {ECO:0000305}.
Subcellular Location	Cytoplasm.
Tissue Specificity	Highly expressed in kidney; moderately expressed in liver; very low expression in brain.