Version 2.78

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

Fully-Specified Name

Component
Isovalerylglycine/Urea
Property
SRto
Time
Pt
System
Plas
Scale
Qn
Method

Additional Names

Short Name
Isovalerylgly/Urea Plas-sRto
Display Name
Isovalerylglycine/Urea (P) [Molar ratio]
Consumer Name Alpha Get Info
Isovalerylglycine/Urea, Blood

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 2.21
Last Updated
Version 2.70
Order vs. Observation
Observation

Language Variants Get Info

Tag Language Translation
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:
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

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

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=48117-6