Reactome | BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA

BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA

Stable Identifier

R-HSA-71261

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

gamma-butyrobetaine + alpha-ketoglutarate + O2 => carnitine + succinate + CO2

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BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA

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Cytosolic gamma-butyrobetaine hydroxylase dimer (BBOX1), a dioxygenase, catalyzes the reaction of oxygen, 4-trimethylammoniobutanoate (TEABT), and 2-oxoglutarate (2OG)to form CO2, succinate (SUCCA), and carnitine (CAR) (Lindstedt and Nordin 1984; Vaz et al. 1998).

Literature References

PubMed ID	Title	Journal	Year
9753662	Carnitine biosynthesis: identification of the cDNA encoding human gamma-butyrobetaine hydroxylase. Vaz, FM, van Gool, S, Ofman, R, Ijlst, L	Biochem Biophys Res Commun	1998
6497835	Multiple forms of gamma-butyrobetaine hydroxylase (EC 1.14.11.1). Lindstedt, S, Nordin, I	Biochem J	1984