48117-6

Isovalerylglycine/Urea [Molar ratio] 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/Urea:SRto:Pt:Plas:Qn:
Long Common Name: Isovalerylglycine/Urea [Molar ratio] in Plasma
Short Name: Isovalerylgly/Urea Plas-sRto
Display Name: Isovalerylglycine/Urea (P) [Molar ratio]
Consumer Name Alpha Get Info: Isovalerylglycine/Urea, Blood

Part Model Get Info

Component
Isovalerylglycine/Urea
LP286298-7
- Analyte
  Isovalerylglycine/Urea
  LP286298-7
  - Component Numerator
    Isovalerylglycine
    LP14516-6
    - Component Numerator Core
      Isovalerylglycine
      LP14516-6
    - Component Numerator Core Suffix
      NULL
  - Component Denominator
    Urea
    LP69843-8
    - Component Denominator Core
      Urea
      LP69843-8
    - Component Denominator Core Suffix
      NULL
- Challenge
  NULL
- Adjustment
  NULL
- Count
  NULL
Property
SRto
LP6868-6
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 2.21
Last Updated: Version 2.70 (MIN)
Order vs. Observation: Observation

Language Variants Get Info

Tag	Language	Translation
cs-CZ	Czech (Czechia)	Izovalerylglycin/močovina:Látkový poměr:Časový bod:Plazma:Kvantitativní:
el-GR	Greek (Greece)	Ισοβαλερυλογλυκίνη/Ουρία:SRto:Pt:Πλάσμα:Qn: Synonyms: CHEM Pt Qn SRto Ισοβαλερυλογλυκίνη Ουρία Πλάσμα
es-ES	Spanish (Spain)	Isovalerilglicina/Urea:Relacion de Sustancia:Punto temporal:Plasma:Qn: Synonyms: Cuantitativo
es-MX	Spanish (Mexico)	Isovalerilglicina / Urea:Proporción de sustancia:Punto temporal:Plasma:Cuantitativo:
fr-CA	French (Canada)	Isovalérylglycine/Urée:Ratio de substance:Temps ponctuel:Plasma:Quantitatif:
fr-FR	French (France)	Isovalérylglycine/urée:Moles ratio:Ponctuel:Plasma:Numérique:
fr-BE	French (Belgium)	Isovalérylglycine/Urée:Ratio de substance:Temps ponctuel:Plasma:Quantitatif:
it-IT	Italian (Italy)	Isovalerilglicina/Urea:Srto:Pt:Plasma:Qn: Synonyms: Chimica Plasma Punto nel tempo (episodio) Rapporto di sostanza
nl-NL	Dutch (Netherlands)	isovalerylglycine/ureum:molaire verhouding:moment:plasma:kwantitatief:
pl-PL	Polish (Poland)	Izowalerianoglicyna/mocznik:współczynnik molowy:punkt w czasie:osocze:ilościowy:
pt-BR	Portuguese (Brazil)	Isovalerilglicina/Uréia:SCrto:Pt:Plasma:Qn: Synonyms: ; Isovalerylgly; sRto; Substance concentration ratio; Point in time; Random; Plasma; Pl; Plsm; Quantitative; QNT; Quant; Quan; Chemistry
ru-RU	Russian (Russian Federation)	Изовалерилглицин/Мочевина:МольОтн:ТчкВрм:Плаз:Колич: Synonyms: Количественный Мольная доля;Молярная доля;Отношение концентраций Плазма Точка во времени;Момент
tr-TR	Turkish (Turkey)	İzovalerilglisin/Üre:SOrn:Zmlı:Plaz:Kant: Synonyms: SubsOranı
zh-CN	Chinese (China)	异戊酰甘氨酸/尿素:物质的量比率:时间点:血浆:定量型: Synonyms: CO(NH2)2 CO(NH2)2;碳酰二胺;脲化学;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
mmol/mmol	Example UCUM Units

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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.