79272-1
3-Hydroxytrimethyl-L-lysine/Trimethyllysine [Molar ratio] in Cerebral spinal fluid
Active
Part Descriptions
LP200265-9 3-Hydroxytrimethyl-L-lysine
3-Hydroxytrimethyl-L-lysine is found within the Lysine degradation (aka L-carnitine biosynthesis) pathway; it is a byproduct of trimethyllysine dioxygenase (trimethyllysine hydroxylase, EC 1.14.11.8), which converts trimethyllysine + 2-oxoglutarate to 3-Hydroxytrimethyl-L-lysine + succinate. Patients with X-linked trimethyllysine hydroxylase (TMLHE) deficiency, a carnitine deficiency disorder associated with autism, have lower than expected levels of carnitine as well as 3-Hydroxytrimethyl-L-lysine, and elevated levels of trimethyllysine. [OMIM: 300777]
Source: Regenstrief LOINC
LP200451-5 Trimethyllysine
Trimethyllysine (N6,N6,N6-Trimethyl-L-lysine) is a methylated derivative of the amino acid lysine. It is a component of histone proteins, a precursor of carnitine and a coenzyme of fatty acid oxidation. N6,N6,N6-Trimethyl-L-lysine residues are found in a number of proteins and are generated by the action of S-adenosyl-L-methionine on exposed lysine residues. When trimethyllysine is released from cognate proteins via proteolysis, it serves as a precursor for carnitine biosynthesis. Mitochondrial 6-N-trimethyllysine dioxygenase converts 6-N-trimethyllysine to 3-hydroxy-6-N-trimethyllysine as the first step for carnitine biosynthesis. Because the subsequent carnitine biosynthesis enzymes are cytosolic, 3-hydroxy-6-N-trimethyllysine must be transported out of the mitochondria by a putative mitochondrial 6-N-trimethyllysine/3-hydroxy-6-N-trimethyllysine transporter system. Plasma -N-trimethyllysine concentrations are significantly lower in systemic carnitine deficiency patients compared to normal individuals, but no significant difference in urinary -N-trimethyllysine excretion is seen between the two groups.[HMDB ID: HMDB01325]
Copyright Copyright ©2005-2009 Genome Alberta (Reference to original publication: Wishart DS, Knox C, Guo AC, et al. HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 37(Database issue):D603-610.)
Source: Human Metabolome Database
Fully-Specified Name
- Component
- 3-Hydroxytrimethyl-L-lysine/Trimethyllysine
- Property
- SRto
- Time
- Pt
- System
- CSF
- Scale
- Qn
- Method
Additional Names
- Short Name
- 3OH-trimethyl-L-lysine/TML CSF-sRto
- Display Name
- 3-Hydroxytrimethyl-L-lysine/Trimethyllysine (CSF) [Molar ratio]
- Consumer Name Alpha Get Info
- 3-Hydroxytrimethyl-L-lysine/Trimethyllysine, Spinal fluid
Basic Attributes
- Class
- CHEM
- Type
- Laboratory
- First Released
- Version 2.54
- Last Updated
- Version 2.54
- Order vs. Observation
- Both
Member of these Panels
| LOINC | Long Common Name |
|---|---|
| 79286-1 | Carnitine biosynthesis intermediates panel - Cerebral spinal fluid |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| es-ES | Spanish (Spain) | 3-Hidroxi-trimetil-lisina/Trimetil lisina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | 3-hidroxitrimetil-L-lisina / trimetilisina: |
| fr-FR | French (France) | 3-hydroxytriméthyl-L-lysine/triméthyllysine: |
| it-IT | Italian (Italy) | 3-Idrossitrimetil-L-lisina/Trimetil-lisina: Synonyms: Chimica Liquido cerebrospinale Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | 3-hydroxytrimethyl-L-lysine/trimethyllysine: |
| tr-TR | Turkish (Turkey) | 3-Hidroksitrimetil-L-lizin/Trimetillizin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | 3-羟基三甲基-L-赖氨酸/三甲基赖氨酸: Synonyms: 2,6-二氨基已酸; |
Example Units
| Unit | Source |
|---|---|
| {ratio} | Example UCUM Units |
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