Reactome | TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA

TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA

Stable Identifier

R-HSA-71241

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

trimethyllysine + alpha-ketoglutarate + O2 => beta-hydroxy-trimethyllysine + succinate + CO2

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TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA

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Trimethyllysine dioxygenase (TMLHE) dimer in the mitochondrial matrix catalyzes the reaction of oxygen, 2-oxoglutarate (2OG), and N6,N6,N6-trimethyl-L-lysine (TMLYS) to form CO2, 3-hydroxy-N6,N6,N6-trimethyl-L-lysine (HTMLYS), and succinate (SUCCA) (Vaz et al. 2001).

Literature References

PubMed ID	Title	Journal	Year
11431483	Molecular and Biochemical Characterization of Rat epsilon -N-Trimethyllysine Hydroxylase, the First Enzyme of Carnitine Biosynthesis. Vaz, FM, Ofman, R, Westinga, K, Back, JW	J Biol Chem	2001