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

Stable Identifier
Reaction [transition]
Homo sapiens
2-oxoglutarate + 4-(trimethylamino)butanoate + O2 = carnitine + CO2 + succinate, gamma-butyrobetaine + alpha-ketoglutarate + O2 => carnitine + succinate + CO2
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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

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
Catalyst Activity

gamma-butyrobetaine dioxygenase activity of BBOX1:AscH-:Fe2+ dimer [cytosol]

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