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
Synonyms
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; 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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
gamma-butyrobetaine dioxygenase activity of BBOX1:AscH-:Fe2+ dimer [cytosol]
Physical Entity
Activity
Orthologous Events