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Proteins/UNIPROT:Q9NVH6
Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML) in the carnitine biosynthesis pathway..
[Isoform 2]: Lacks trimethyllysine dioxygenase activity..
[Isoform 3]: Lacks trimethyllysine dioxygenase activity..
[Isoform 4]: Lacks trimethyllysine dioxygenase activity..
[Isoform 5]: Lacks trimethyllysine dioxygenase activity..
Function from UniProtKB (Q9NVH6), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
14 statements
14 statements · 5 reference databases · Subject: TMLHE · Sources: Hetionet, Human-GEM, Reactome, Rhea, UniProt
2 reactions
2-oxoglutarate(2-)+N(6),N(6),N(6)-trimethyl-L-lysine+dioxygen3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine zwitterion+carbon dioxide+succinate(2-)
Catalysed by TMLHE
Reaction pageLeft to rightBalancedEC 1.14.11.8cytosol
Human-GEM MAR06979CC BY 4.0Reference data
N(6),N(6),N(6)-trimethyl-L-lysine+2-oxoglutarate(2-)+dioxygen(3S)-3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine
7 names
Resolves to: TMLHE
Catalysed by TMLHE
Reaction pageDirection not assertedBalanced
Rhea RHEA:14181CC BY 4.0Reference data