BBOX1 polyclonal antibody

BBOX1 (butyrobetaine (γ), 2-oxoglutarate dioxygenase 1), also known as BBH, BBOX, G-BBH (γ-butyrobetaine hydroxylase) or γ-BBH, is a member of the γ-BBH/TMLD family of proteins. Localizing to the cytoplasm and predominantly expressed in kidney, BBOX1 plays a role in the biosynthesis of amines, polyamines and carnitines. More specifically, BBOX1 catalyzes the hydroxylation of γ-butyrobetaine to L-carnitine, a reaction that requires iron and ascorbate as cofactors. This is the last of five steps comprising the L-carnitine biosynthesis pathway and it is important for the proper transport of activated fatty acids across the mitochondrial membrane. Coupled to this reaction, BBOX1 simultaneously catalyzes the oxidative decarboxylation of α-ketoglutarate to succinate.