Reaction: BBOX1:AscH-:Fe2+ dimer dioxygenates TEABT and 2OG to form CAR and SUCCA
- in pathway: Carnitine synthesis
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).
Reaction - small molecule participants:
CAR [cytosol]
SUCCA [cytosol]
CO2 [cytosol]
2OG [cytosol]
TEABT [cytosol]
O2 [cytosol]
Reactome.org reaction link: R-HSA-71261
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Reaction input - small molecules:
2-oxoglutarate(2-)
4-(trimethylammonio)butanoate
dioxygen
Reaction output - small molecules:
carnitine
succinate(2-)
carbon dioxide
Reactome.org link: R-HSA-71261