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Literature summary for 1.14.11.8 extracted from
- Human carnitine biosynthesis proceeds via (2S,3S)-3-hydroxy-Nepsilon-trimethyllysine (2017), Chem. Commun. (Camb.), 53, 440-442 .
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| recombinant expression of the enzyme fused to MBP | Homo sapiens |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| mitochondrion | - |
Homo sapiens | 5739 | - |
Metals/Ions
| Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|
| Fe2+ | dependent on, required for catalysis | Homo sapiens |  |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 | Homo sapiens | - |
(3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | Q9NVH6 | - |
- |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| recombinant MBP-taged enzyme by affinity chromatography, tag cleavage by rTEV protease, and gel filtration | Homo sapiens |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 | - |
Homo sapiens | (3S)-3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| Nepsilon-trimethyllysine hydroxylase | - |
Homo sapiens |
| TMLH | - |
Homo sapiens |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 7.5 | - |
- |
Homo sapiens |
General Information
| General Information | Comment | Organism |
|---|---|---|
| evolution | the enzyme belongs to the family of 2-oxoglutarate (2OG)-dependent oxygenases | Homo sapiens |
| 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 | Homo sapiens |
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Release 2023.1 (January 2023)
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