Gamma-butyrobetaine dioxygenase

In enzymology, a gamma-butyrobetaine dioxygenase is an enzyme that catalyzes the chemical reaction

4-trimethylammoniobutanoate + 2-oxoglutarate + O2 rightleftharpoons 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2

The 3 substrates of this enzyme are 4-trimethylammoniobutanoate, 2-oxoglutarate, and O2, whereas its 3 products are 3-hydroxy-4-trimethylammoniobutanoate, succinate, and CO2.

This enzyme belongs to the family of oxidoreductases, specifically those acting on paired donors, with O2 as oxidant and incorporation or reduction of oxygen. The oxygen incorporated need not be derived from O2 with 2-oxoglutarate as one donor, and incorporation of one atom o oxygen into each donor. The systematic name of this enzyme class is 4-trimethylammoniobutanoate,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating). Other names in common use include alpha-butyrobetaine hydroxylase, gamma-butyrobetaine hydroxylase, and butyrobetaine hydroxylase. This enzyme participates in lysine degradation. It has 2 cofactors: iron, and Ascorbate.


  • Lindstedt G, Lindstedt S "Cofactor requirements of gamma-butyrobetaine hydroxylase from rat liver". J. Biol. Chem. 245 4178–86.

External links

The CAS registry number for this enzyme class is .

Gene Ontology (GO) codes

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