1.14.11.8: trimethyllysine dioxygenase
This is an abbreviated version!
For detailed information about trimethyllysine dioxygenase, go to the full flat file.
Word Map on EC 1.14.11.8
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1.14.11.8
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carnitine
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gamma-butyrobetaine
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autism
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inborn
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proliferator-activated
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hydroxymethyltransferase
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transporter-2
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aldolase
- 1.14.11.8
- carnitine
- gamma-butyrobetaine
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autism
-
inborn
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proliferator-activated
- hydroxymethyltransferase
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transporter-2
- aldolase
Reaction
Synonyms
6-N-trimethyllysine dioxygenase, epsilon-N-trimethyllysine hydroxylase, epsilon-trimethyllysine 2-oxoglutarate dioxygenase, eta-N-trimethyllysine hydroxylase, Nepsilon-trimethyllysine hydroxylase, oxygenase, trimethyllysine di-, TML dioxygenase, TML hydroxylase, TML-alpha-ketoglutarate dioxygenase, TMLD, TMLH, TMLH-a, TMLH-b, trimethyllysine alpha-ketoglutarate dioxygenase, trimethyllysine hydroxylase
ECTree
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General Information
General Information on EC 1.14.11.8 - trimethyllysine dioxygenase
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evolution
metabolism
physiological function
additional information
the enzyme belongs to the family of 2-oxoglutarate (2OG)-dependent oxygenases
evolution
the enzyme belongs to the family of 2-oxoglutarate (2OG)-dependent oxygenases. Members of Fe(II) and 2-oxoglutarate-dependent oxygenases catalyze stereoselective hydroxylations of unactivated C-H bonds in various (bio)molecules, including proteins, DNA, and small molecule metabolites
evolution
the enzyme belongs to the non-heme Fe(II) and 2-oxoglutarate dependent oxygenases
the enzyme catalyzes the first step in the biosynthesis of carnitine
metabolism
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the enzyme catalyzes the first step in the biosynthesis of carnitine, i.e. L-3-hydroxy-4-N-N-N-trimethylaminobutyrate. Lysine in protein peptide linkages undergoes methylation of the 1-amino group to yield trimethyllysine, TML, which is released upon protein degradation. Muscle is the major source of TML. The released TML is further oxidised to gamma-butyrobetaine by the action of trimethyllysine dioxygenase, 3-hydroxy-N-trimethyllysine aldolase and 4-N-trimethylaminobutyraldehyde dehydrogenase
metabolism
the enzyme catalyses the first step in mammalian biosynthesis of carnitine, which plays a crucial role in fatty acid metabolism. Carnitine biosynthesis patway, overview
metabolism
trimethyllysine hydroxylase (TMLH) catalyses C-3 hydroxylation of Nepsilon-trimethyllysine in the first step of carnitine biosynthesis
metabolism
trimethyllysine hydroxylase (TMLH) catalyses C-3 hydroxylation of Nepsilon-trimethyllysine in the first step of carnitine biosynthesis in humans
metabolism
trimethyllysine hydroxylase (TMLH) catalyses the first step in carnitine biosynthesis, the conversion of N6,N6,N6-trimethyl-L-lysine to 3-hydroxy-N6,N6,N6-trimethyl-L-lysine
metabolism
trimethyllysine hydroxylase (TMLH) is involved in the first step of the physiologically important carnitine biosynthesis pathway. The enzyme catalyzes C-3 hydroxylation of (2S)-Nepsilon-L-trimethyllysine, to produce 3-hydroxy-Nepsilon-L-trimethyllysine, which then undergoes three additional enzymatic steps to the final L-carnitine
trimethyllysine hydroxylase (TMLH) catalyses C-3 hydroxylation of Nepsilon-trimethyllysine in the first step of carnitine biosynthesis
physiological function
trimethyllysine hydroxylase (TMLH) catalyses C-3 hydroxylation of Nepsilon-trimethyllysine in the first step of carnitine biosynthesis in humans
physiological function
the enzyme catalyzes the first step in carnitine biosynthesis
residue Asp131 in TMLHis responsible for specific binding of the alpha-ammonium group of trimethyllysine
additional information
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residue Asp131 in TMLHis responsible for specific binding of the alpha-ammonium group of trimethyllysine