79313-3
Gamma butyrobetaine/Trimethyllysine [Molar ratio] in Urine
Active
Part Descriptions
LP200429-1 Gamma butyrobetaine
Gamma-butyrobetaine dioxygenase catalyzes the formation of L-carnitine from gamma-butyrobetaine (G-butyrobetaine). G-butyrobetaine levels are altered in various carnitine biosynthesis disorders, including X-linked trimethyllysine hydroxylase epsilon (TMLHE) deficiency. Decreased levels of gamma-butyrobetaine and 3-hydroxy-6-N-trimethyllysine along with increased levels of 6-N-trimethyllysine in plasma and urine were found in a patient with a deletion of exon 2 of the trimethyllysine hydroxylase epsilon (TMLHE) gene. PMID: 22566635
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
- Gamma butyrobetaine/Trimethyllysine
- Property
- SRto
- Time
- Pt
- System
- Urine
- Scale
- Qn
- Method
Additional Names
- Short Name
- G-butyrobetaine/TML Ur-sRto
- Display Name
- Gamma butyrobetaine/Trimethyllysine (U) [Molar ratio]
- Consumer Name Alpha Get Info
- Gamma butyrobetaine/Trimethyllysine, Urine
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 |
|---|---|
| 79287-9 | Carnitine biosynthesis intermediates panel - Urine |
Language Variants Get Info
| Tag | Language | Translation |
|---|---|---|
| es-ES | Spanish (Spain) | Gamma butirobetaina/Trimetil lisina: Synonyms: Cuantitativo |
| es-MX | Spanish (Mexico) | Gamma butirobetaína / trimetilisina: |
| fr-FR | French (France) | Gamma butyrobétaïne/triméthyllysine: |
| it-IT | Italian (Italy) | Gsmma butirro-betaina/Trimetil-lisina: Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza |
| nl-NL | Dutch (Netherlands) | gamma butyrobetaïne/trimethyllysine: |
| tr-TR | Turkish (Turkey) | Gamma butirobetain/Trimetillizin: Synonyms: SubsOranı |
| zh-CN | Chinese (China) | Gamma 丁酰甜菜碱/三甲基赖氨酸: Synonyms: Gamma-丁酰甜菜碱; |
Example Units
| Unit | Source |
|---|---|
| {ratio} | Example UCUM Units |
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LOINC Copyright
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