Version 2.76

Status Information

Status
DISCOURAGED
Comment
No units are associated with this term - cannot verify validity

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
Property
MCnc
Time
Pt
System
Urine
Scale
Qn
Method

Additional Names

Short Name
Isovalerylgly Ur-mCnc
Display Name
Isovalerylglycine (U) [Mass/Vol]
Consumer Name Alpha Get Info
Isovalerylglycine, Urine

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 1.0j-a
Last Updated
Version 2.44
Order vs. Observation
Observation

Language Variants Get Info

Tag Language Translation
es-AR Spanish (Argentina) isovalerilglicina:concentración de masa:punto en el tiempo:orina:cuantitativo:
es-ES Spanish (Spain) Isovalerilglicina:Concentración de masa:Punto temporal:Orina:Qn:
Synonyms: Cuantitativo
es-MX Spanish (Mexico) Isovalerilglicina:Concentración de masa:Punto temporal:Orina:Cuantitativo:
et-EE Estonian (Estonia) Isovalerüülglütsiin:MCnc:Pt:U:Qn:
Synonyms: Juhuslik Kvantitatiivne Uriin
fr-CA French (Canada) Isovalérylglycine:Concentration de masse:Temps ponctuel:Urine:Quantitatif:
fr-BE French (Belgium) Isovalérylglycine:Concentration de masse:Temps ponctuel:Urine:Quantitatif:
it-IT Italian (Italy) Isovalerilglicina:MCnc:Pt:Urine:Qn:
Synonyms: Chimica Concentrazione di Massa Punto nel tempo (episodio)
ko-KR Korean (Korea, Republic Of) 이소발레릴글리신:질량농도:검사시점:뇨:정량:
nl-NL Dutch (Netherlands) isovalerylglycine:massa/volume:moment:urine:kwantitatief:
pt-BR Portuguese (Brazil) Isovalerilglicina:MCnc:Pt:Urina:Qn:
Synonyms: ; Isovalerylgly; Mass concentration; Level; Point in time; Random; Ur; UR; Urn; UA; Quantitative; QNT; Quant; Quan; Chemistry
ru-RU Russian (Russian Federation) Изовалерилглицин:МассКонц:ТчкВрм:Моча:Колич:
Synonyms: Количественный Массовая концентрация Точка во времени;Момент
tr-TR Turkish (Turkey) İzovalerilglisin:KütlKons:Zmlı:İdrar:Kant:
zh-CN Chinese (China) 异戊酰甘氨酸:质量浓度:时间点:尿液:定量型:
Synonyms: 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 水平;浓度;质量浓度(单位体积)

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