55930-2

Isovalerylglycine [Moles/volume] in Plasma

Active

Part Description

LP14516-6 Isovalerylglycine
Isovalerylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine (IVG). It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results. PMID: 17850781. 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, Isovalerylglycine

LOINC Names Get Info

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

Part Model Get Info

Component
Isovalerylglycine
LP14516-6
- Analyte
  Isovalerylglycine
  LP14516-6
  - Component Numerator
    Isovalerylglycine
    LP14516-6
    - Component Numerator Core
      Isovalerylglycine
      LP14516-6
    - 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
- Time Core
  Pt
  LP6960-1
- Time Modifier
  NULL
System
Plas
LP7479-1
- System Core
  Plas
  LP7479-1
- Super System
  NULL
Scale
Qn
LP7753-9
Method
NULL

Basic Attributes

Class: CHEM
Type: Laboratory
First Released: Version 2.29
Last Updated: Version 2.70 (MIN)
Order vs. Observation: Both

Language Variants Get Info

Tag	Language	Translation
cs-CZ	Czech (Czechia)	Izovalerylglycin:Látková koncentrace:Časový bod:Plazma:Kvantitativní:
el-GR	Greek (Greece)	Ισοβαλερυλογλυκίνη:SCnc:Pt:Πλάσμα:Qn: Synonyms: CHEM Pt Qn SCnc Ισοβαλερυλογλυκίνη Πλάσμα
es-ES	Spanish (Spain)	Isovalerilglicina:Concentración de sustancia:Punto temporal:Plasma:Qn: Synonyms: Cuantitativo
es-MX	Spanish (Mexico)	Isovalerilglicina:Concentración de sustancias:Punto temporal:Plasma:Cuantitativo:
et-EE	Estonian (Estonia)	Isovalerüülglütsiin:SCnc:Pt:P:Qn: Synonyms: Aine kontsentratsioon Juhuslik Kvantitatiivne Plasma
fr-CA	French (Canada)	Isovalérylglycine:Concentration de substance:Temps ponctuel:Plasma:Quantitatif:
fr-FR	French (France)	Isovalérylglycine:Moles/Volume:Ponctuel:Plasma:Numérique:
fr-BE	French (Belgium)	Isovalérylglycine:Concentration de substance:Temps ponctuel:Plasma:Quantitatif:
it-IT	Italian (Italy)	Isovalerilglicina:SCnc:Pt:Plasma:Qn: Synonyms: Chimica Concentrazione di Sostanza Plasma Punto nel tempo (episodio)
nl-NL	Dutch (Netherlands)	isovalerylglycine:mol/volume:moment:plasma:kwantitatief:
pl-PL	Polish (Poland)	Izowalerianoglicyna:stężenie molowe:punkt w czasie:osocze:ilościowy:
pt-BR	Portuguese (Brazil)	Isovalerilglicina:#N/A:Pt:Plasma:Qn: Synonyms: ; Isovalerylgly; Substance concentration; Level; Point in time; Random; Plasma; Pl; Plsm; Quantitative; QNT; Quant; Quan; Chemistry
ru-RU	Russian (Russian Federation)	Изовалерилглицин:МолКонц:ТчкВрм:Плаз:Колич: Synonyms: Количественный Молярность;Молярная объёмная концентрация Плазма Точка во времени;Момент
tr-TR	Turkish (Turkey)	İzovalerilglisin:SubKons:Zmlı:Plaz:Kant:
zh-CN	Chinese (China)	异戊酰甘氨酸:物质的量浓度:时间点:血浆:定量型: Synonyms: 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

Example Units

Unit	Source
mmol/L	Example UCUM Units

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LOINC Copyright

Copyright © Regenstrief Institute, Inc. All Rights Reserved. To the extent included herein, the LOINC table and LOINC codes are copyright © Regenstrief Institute, Inc. and the Logical Observation Identifiers Names and Codes (LOINC) Committee. See https://loinc.org/license for the full LOINC copyright and license.