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

2-oxoglutarate + N(6),N(6),N(6)-trimethyl-L-lysine + O2 => (3S)-3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine + CO2 + succinate, trimethyllysine + alpha-ketoglutarate + O2 => beta-hydroxy-trimethyllysine + succinate + CO2

ReviewStatus

5/5

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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) (Monfregola et al. 2005; 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
15754339	Functional analysis of TMLH variants and definition of domains required for catalytic activity and mitochondrial targeting Vaz, FM, D'Urso, M, Arbucci, S, Monfregola, J, Terracciano, A, Ursini, MV, Wanders, RJA, van Vlies, N, Cevenini, A	J Cell Physiol	2005