Version 2.78

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
Urine
Scale
Qn
Method

Additional Names

Short Name
3OH-trimethyl-L-lysine/TML Ur-sRto
Display Name
3-Hydroxytrimethyl-L-lysine/Trimethyllysine (U) [Molar ratio]
Consumer Name Alpha Get Info
3-Hydroxytrimethyl-L-lysine/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) 3-Hidroxi-trimetil-lisina/Trimetil lisina:Relacion de Sustancia:Punto temporal:Orina:Qn:
Synonyms: Cuantitativo
es-MX Spanish (Mexico) 3-hidroxitrimetil-L-lisina / trimetilisina:Proporción de sustancia:Punto temporal:Orina:Cuantitativo:
fr-FR French (France) 3-hydroxytriméthyl-L-lysine/triméthyllysine:Moles ratio:Ponctuel:Urine:Numérique:
it-IT Italian (Italy) 3-Idrossitrimetil-L-lisina/Trimetil-lisina:Srto:Pt:Urine:Qn:
Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza
nl-NL Dutch (Netherlands) 3-hydroxytrimethyl-L-lysine/trimethyllysine:molaire verhouding:moment:urine:kwantitatief:
tr-TR Turkish (Turkey) 3-Hidroksitrimetil-L-lizin/Trimetillizin:SOrn:Zmlı:İdrar:Kant:
Synonyms: SubsOranı
zh-CN Chinese (China) 3-羟基三甲基-L-赖氨酸/三甲基赖氨酸:物质的量比率:时间点:尿液:定量型:
Synonyms: 2,6-二氨基已酸;Lys;氨基已氨酸;溶细胞素 Ⅲ III 三 化学;化学检验项目;化学检验项目类;化学类;化学试验;非刺激耐受型化学检验项目;非刺激耐受型化学检验项目类;非刺激耐受型化学试验;非刺激耐受型化学试验类 叁 可用数量表示的;定量性;数值型;数量型;连续数值型标尺 尿;小便;下泉 左 左侧 左侧（Left，L） 时刻;随机;随意;瞬间 物质的量比值;物质的量比;摩尔比;摩尔比率;摩尔比值

Example Units

Unit Source
{ratio} Example UCUM Units

LOINC Terminology Service (API) using HL7® FHIR® Get Info

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=79275-4