Version 2.80

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
Bld.dot
Scale
Qn
Method

Additional Names

Long Common Name
3-Hydroxytrimethyl-L-lysine/Trimethyllysine [Molar ratio] in DBS
Short Name
3OH-trimethyl-L-lysine/TML DBS-sRto
Display Name
3-Hydroxytrimethyl-L-lysine/Trimethyllysine (DBS) [Molar ratio]
Consumer Name Alpha Get Info
3-Hydroxytrimethyl-L-lysine/Trimethyllysine, Dried blood spot

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 2.54
Last Updated
Version 2.61 (MIN)
Order vs. Observation
Both

Member of these Panels

LOINC Long Common Name
79284-6 Carnitine biosynthesis intermediates panel - DBS

Language Variants Get Info

Tag Language Translation
el-GR Greek (Greece) 3-Υδροξυτριμεθυλο-L-λυσίνη/Τριμεθυλολυσίνη:SRto:Pt:Bld.dot:Qn:
Synonyms: 3-Υδροξυτριμεθυλο-L-λυσίνη SRto Λυσίνη Τριμεθυλολυσίνη
es-ES Spanish (Spain) 3-Hidroxi-trimetil-lisina/Trimetil lisina:Relacion de Sustancia:Punto temporal:gota de sangre (papel de filtro):Qn:
Synonyms: Cuantitativo
es-MX Spanish (Mexico) 3-hidroxitrimetil-L-lisina / trimetilisina:Proporción de sustancia:Punto temporal:DBS:Cuantitativo:
fr-FR French (France) 3-hydroxytriméthyl-L-lysine/triméthyllysine:Moles ratio:Ponctuel:Sang buvard:Numérique:
it-IT Italian (Italy) 3-Idrossitrimetil-L-lisina/Trimetil-lisina:Srto:Pt:Sangue.su carta da filtro:Qn:
Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza Sangue Spot sangue secco
nl-NL Dutch (Netherlands) 3-hydroxytrimethyl-L-lysine/trimethyllysine:molaire verhouding:moment:gedroogde bloedspot:kwantitatief:
tr-TR Turkish (Turkey) 3-Hidroksitrimetil-L-lizin/Trimetillizin:SOrn:Zmlı:Kan.nokta: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:全血.斑点:定量型:
Synonyms: 2,6-二氨基已酸;Lys;氨基已氨酸;溶细胞素 Ⅲ III 三 全血斑点(滤纸);滤纸;血液.斑点;血液斑点(滤纸) 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 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 血;血液

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=79273-9