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

2-oxoglutarate + 4-(trimethylamino)butanoate + O2 = carnitine + CO2 + succinate, 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; Tars et al. 2010; Vaz et al. 1998).

Literature References

PubMed ID	Title	Journal	Year
6497835	Multiple forms of gamma-butyrobetaine hydroxylase (EC 1.14.11.1). Nordin, I, Lindstedt, S	Biochem J	1984
20599753	Crystal structure of human gamma-butyrobetaine hydroxylase Kuka, J, Kotelovica, S, Rumnieks, J, Viksna, A, Tars, K, Kazaks, A, Zeltins, A, Dambrova, M, Leonciks, A, Sharipo, J, Liepinsh, E	Biochem Biophys Res Commun	2010
9753662	Carnitine biosynthesis: identification of the cDNA encoding human gamma-butyrobetaine hydroxylase. Vaz, FM, Ijlst, L, van Gool, S, Ofman, R	Biochem Biophys Res Commun	1998