Reaction: TMLHE dimer dioxygenates TMLYS and 2OG to form HTMLYS and SUCCA
- in pathway: Carnitine synthesis
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) (Monfregola et al. 2005; Vaz et al. 2001).
Reaction - small molecule participants:
HTMLYS [mitochondrial matrix]
SUCCA [mitochondrial matrix]
CO2 [mitochondrial matrix]
O2 [mitochondrial matrix]
2OG [mitochondrial matrix]
TMLYS [mitochondrial matrix]
Reactome.org reaction link: R-HSA-71241
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Reaction input - small molecules:
dioxygen
2-oxoglutarate(2-)
N(6),N(6),N(6)-trimethyl-L-lysine zwitterion
Reaction output - small molecules:
(3S)-3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine(1+)
succinate(2-)
carbon dioxide
Reactome.org link: R-HSA-71241