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

Stable Identifier
R-HSA-71241
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
trimethyllysine + alpha-ketoglutarate + O2 => beta-hydroxy-trimethyllysine + succinate + CO2
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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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
trimethyllysine dioxygenase activity of TMLHE:AscH-:Fe2+ dimer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events