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Part Description

LP268082-7   Globotriaosylsphingosine
Globotriaosylsphingosine (lyso-Gb3) is the deacylated, cationic, amphiphilic glycolipid product of globotriaosylceramide (Gb3). Gb3, lyso-Gb3, and an un-described additional metabolite of Gb3 are markedly increased in the plasma of male patients with classic Fabry disease. Fabry disease is caused by a deficiency of alpha-galactosidase A due to a mutation in the alpha-galactosidase A gene on the X chromosome. It leads to renal insufficiency, cardiac hypertrophy, and neuropathy that is thought to be due to the accumulation of Gb3 in the endothelium. However, treatment of Fabry disease with enzyme therapy decreases the Gb3 in the endothelium, but does not eliminate disease symptoms to the degree expected. In addition, plasma and urinary levels of Gb3 do not correlate with disease severity which suggests little or no correlation of the plasma level of Gb3 and the cause of disease. The plasma levels that do correlate with symptoms of disease are lyso-Gb3. Lyso-Gb3 is present in the plasma of Fabry patients in much higher levels than Gb3. It is believed to inhibit the activity of alpha-galactosidase A, promote the storage of Gb3, and induce the proliferation of smooth muscle cells in vitro. In Fabry disease, there is known accelerated hypertrophy of the walls of the radial artery and a thickening of the intima media of the common carotid artery as a result of smooth muscle cell proliferation. PMID: 18287059 Source: Regenstrief LOINC

LOINC Names Get Info

Fully-Specified Name
Globotriaosylsphingosine:SCnc:Pt:Bld:Qn:
Long Common Name
Globotriaosylsphingosine [Moles/volume] in Blood
Short Name
Globotriaosylsphingosine Bld-sCnc
Display Name
Globotriaosylsphingosine (Bld) [Moles/Vol]
Consumer Name Alpha Get Info
Globotriaosylsphingosine, Blood

Part Model Get Info

  • Component
    Globotriaosylsphingosine
    LP268082-7
    • Analyte
      Globotriaosylsphingosine
      LP268082-7
      • Component Numerator
        Globotriaosylsphingosine
        LP268082-7
        • Component Numerator Core
          Globotriaosylsphingosine
          LP268082-7
        • Component Numerator Core Suffix
          NULL
           
      • Component Denominator
        NULL
         
        • Component Denominator Core
          NULL
           
        • Component Denominator Core Suffix
          NULL
           
    • Challenge
      NULL
       
    • Adjustment
      NULL
       
    • Count
      NULL
       
  • Property
    SCnc
    LP6860-3
  • Time
    Pt
    LP6960-1
  • System
    Bld
    LP7057-5
    • System Core
      Bld
      LP7057-5
    • Super System
      NULL
       
  • Scale
    Qn
    LP7753-9
  • Method
    NULL
     

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 2.66
Last Updated
Version 2.66 (ADD)
Order vs. Observation
Observation

Member of these Panels

LOINCLong Common Name
92744-2Hepatosplenomegaly due to lysosomal storage disorders screening panel - Blood

Language Variants Get Info

TagLanguageTranslation
cs-CZCzech (Czechia)Globotriaosylsfingosin:Látková koncentrace:Časový bod:Krev:Kvantitativní:
el-GRGreek (Greece)Σφαιροτριαοσυλσφιγγοσίνη:SCnc:Pt:Αίμα:Qn:
Synonyms: CHEM Pt Qn SCnc Αίμα Σφαιροτριαοσυλσφιγγοσίνη
es-ESSpanish (Spain)Globotriaosilesfingosina:Concentración de sustancia:Punto temporal:Sangre:Qn:
Synonyms: Cuantitativo
es-MXSpanish (Mexico)Globotriaosilsfingosina:Concentración de sustancias:Punto temporal:Sangre:Cuantitativo:
fr-FRFrench (France)Globotriaosylsphingosine:Moles/Volume:Ponctuel:Sang:Numérique:
it-ITItalian (Italy)Globotriaosylsphingosine:SCnc:Pt:Sangue:Qn:
Synonyms: Chimica Concentrazione di Sostanza Punto nel tempo (episodio) Sangue
pl-PLPolish (Poland)Globotriaozylosfingozyna:stężenie molowe:punkt w czasie:krew:ilościowy:
zh-CNChinese (China)三聚己糖神经鞘氨醇:物质的量浓度:时间点:全血:定量型:
Synonyms: 三聚己糖鞘氨醇;三己糖基神经鞘氨醇;三糖基神经鞘氨醇;Gb3;Globotriaosyl ceramide;三糖基神经酰胺;三糖基 N-脂酰鞘氨醇;三聚己糖神经酰胺;三己糖基神经酰胺 克分子浓度;克分子浓度(单位体积);摩尔浓度;摩尔浓度(单位体积);物质的量浓度(单位体积) 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 时刻;随机;随意;瞬间 血;血液

Example Units

UnitSource
nmol/mLExample UCUM Units

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

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=92753-3