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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

LOINC Names Get Info

Fully-Specified Name
Gamma butyrobetaine/Trimethyllysine:SRto:Pt:Urine:Qn:
Long Common Name
Gamma butyrobetaine/Trimethyllysine [Molar ratio] in Urine
Short Name
G-butyrobetaine/TML Ur-sRto
Display Name
Gamma butyrobetaine/Trimethyllysine (U) [Molar ratio]
Consumer Name Alpha Get Info
Gamma butyrobetaine/Trimethyllysine, Urine

Part Model Get Info

  • Component
    Gamma butyrobetaine/Trimethyllysine
    LP286069-2
    • Analyte
      Gamma butyrobetaine/Trimethyllysine
      LP286069-2
      • Component Numerator
        Gamma butyrobetaine
        LP200429-1
        • Component Numerator Core
          Gamma butyrobetaine
          LP200429-1
        • Component Numerator Core Suffix
          NULL
           
      • Component Denominator
        Trimethyllysine
        LP200451-5
        • Component Denominator Core
          Trimethyllysine
          LP200451-5
        • Component Denominator Core Suffix
          NULL
           
    • Challenge
      NULL
       
    • Adjustment
      NULL
       
    • Count
      NULL
       
  • Property
    SRto
    LP6868-6
  • Time
    Pt
    LP6960-1
  • System
    Urine
    LP7681-2
    • System Core
      Urine
      LP7681-2
    • Super System
      NULL
       
  • Scale
    Qn
    LP7753-9
  • Method
    NULL
     

Basic Attributes

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

Member of these Panels

LOINCLong Common Name
79287-9Carnitine biosynthesis intermediates panel - Urine

Language Variants Get Info

TagLanguageTranslation
cs-CZCzech (Czechia)Gama-butyrobetain/trimethyllysin:Látkový poměr:Časový bod:Moč:Kvantitativní:
el-GRGreek (Greece)γ-βουτυροβεταΐνη/Τριμεθυλολυσίνη:SRto:Pt:Ούρα:Qn:
Synonyms: CHEM Pt Qn SRto γ-βουτυροβεταΐνη Ούρα Τριμεθυλολυσίνη
es-ESSpanish (Spain)Gamma butirobetaina/Trimetil lisina:Relacion de Sustancia:Punto temporal:Orina:Qn:
Synonyms: Cuantitativo
es-MXSpanish (Mexico)Gamma butirobetaína / trimetilisina:Proporción de sustancia:Punto temporal:Orina:Cuantitativo:
fr-FRFrench (France)Gamma butyrobétaïne/triméthyllysine:Moles ratio:Ponctuel:Urine:Numérique:
it-ITItalian (Italy)Gsmma butirro-betaina/Trimetil-lisina:Srto:Pt:Urine:Qn:
Synonyms: Chimica Punto nel tempo (episodio) Rapporto di sostanza
nl-NLDutch (Netherlands)gamma butyrobetaïne/trimethyllysine:molaire verhouding:moment:urine:kwantitatief:
pl-PLPolish (Poland)Gamma butyrobetaina/trimetylolizyna:współczynnik molowy:punkt w czasie:mocz:ilościowy:
tr-TRTurkish (Turkey)Gamma butirobetain/Trimetillizin:SOrn:Zmlı:İdrar:Kant:
Synonyms: SubsOranı
zh-CNChinese (China)Gamma 丁酰甜菜碱/三甲基赖氨酸:物质的量比率:时间点:尿液:定量型:
Synonyms: Gamma-丁酰甜菜碱;γ-丁酰甜菜碱;Gamma-丁酰基甜菜碱;γ-丁酰基甜菜碱;γ-丁基甜菜碱;γ-三甲铵基丁内盐;γ-丁内铵盐;γ-三甲氨基丁内盐 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 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 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

UnitSource
{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=79313-3