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

Stable Identifier
R-HSA-71241
Type
Reaction
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.

Back, JW, Vaz, FM, Westinga, K, Ofman, R

J Biol Chem 2001
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Catalyst Activity

trimethyllysine dioxygenase activity of TMLHE:AscH-:Fe2+ dimer [mitochondrial matrix]

Physical Entity
TMLHE:AscH-:Fe2+ dimer [mitochondrial matrix] (Homo sapiens)
Activity
trimethyllysine dioxygenase activity (GO:0050353)
Orthologous Events
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Caenorhabditis elegans)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Canis familiaris)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Danio rerio)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Drosophila melanogaster)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Gallus gallus)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Mus musculus)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Rattus norvegicus)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Sus scrofa)
TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA (Xenopus tropicalis)
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