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
2-oxoglutarate + 4-(trimethylamino)butanoate + O2 = carnitine + CO2 + succinate, gamma-butyrobetaine + alpha-ketoglutarate + O2 => carnitine + succinate + CO2
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
Participants
Participates
Catalyst Activity

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

Orthologous Events
Cite Us!