IUBMB Enzyme Nomenclature


Accepted name: trimethyllysine dioxygenase

Reaction: N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 = 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2

Other name(s): trimethyllysine α-ketoglutarate dioxygenase; TML-α-ketoglutarate dioxygenase; TML hydroxylase

Systematic name: N6,N6,N6-trimethyl-L-lysine,2-oxoglutarate:oxygen oxidoreductase (3-hydroxylating)

Comments: Requires Fe2+ and ascorbate.

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, CAS registry number: 74622-49-4


1. Hulse, J.D., Ellis, S.R. and Henderson, L.M. Carnitine biosynthesis. β-Hydroxylation of trimethyllysine by an α-ketoglutarate-dependent mitochondrial dioxygenase. J. Biol. Chem. 253 (1978) 1654-1659. [PMID: 627563]

[EC created 1983]

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