54308-2

Isovalerylglycine [Moles/volume] in Cerebrospinal Fluid

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:CSF:Qn:
Long Common Name: Isovalerylglycine [Moles/volume] in Cerebrospinal Fluid
Short Name: Isovalerylgly CSF-sCnc
Display Name: Isovalerylglycine (CSF) [Moles/Vol]
Consumer Name Alpha Get Info: Isovalerylglycine, Spinal fluid

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
CSF
LP7156-5
- System Core
  CSF
  LP7156-5
- Super System
  NULL
Scale
Qn
LP7753-9
Method
NULL

Basic Attributes

Class: CHEM
Type: Laboratory
First Released: Version 2.27
Last Updated: Version 2.83 (MIN)
Order vs. Observation: Both

Language Variants Get Info

Tag	Language	Translation
cs-CZ	Czech (Czechia)	Izovalerylglycin:Látková koncentrace:Časový bod:Mozkomíšní mok:Kvantitativní:
de-DE	German (Germany)	Isovalerylglycin:Stoffmengenkonzentration:Zeitpunkt:Liquor:Quantitativ: Synonyms: Zerebrospinalflüssigkeit
el-GR	Greek (Greece)	Ισοβαλερυλογλυκίνη:SCnc:Pt:ΕΝΥ:Qn: Synonyms: CHEM Pt Qn SCnc ΕΝΥ Ισοβαλερυλογλυκίνη
es-ES	Spanish (Spain)	Isovalerilglicina:Concentración de sustancia:Punto temporal:Líquido Cefalorraquideo:Qn: Synonyms: Cuantitativo
es-MX	Spanish (Mexico)	Isovalerilglicina:Concentración de sustancias:Punto temporal:Fluído espinal cerebral:Cuantitativo:
et-EE	Estonian (Estonia)	Isovalerüülglütsiin:SCnc:Pt:CSF:Qn: Synonyms: Aine kontsentratsioon Juhuslik Kvantitatiivne Liikvor
fr-CA	French (Canada)	Isovalérylglycine:Concentration de substance:Temps ponctuel:LCR:Quantitatif:
fr-FR	French (France)	Isovalérylglycine:Moles/Volume:Ponctuel:Liquide céphalorachidien:Numérique:
fr-BE	French (Belgium)	Isovalérylglycine:Concentration de substance:Temps ponctuel:LCR:Quantitatif:
it-IT	Italian (Italy)	Isovalerilglicina:SCnc:Pt:LCS:Qn: Synonyms: Chimica Concentrazione di Sostanza Liquido cerebrospinale Punto nel tempo (episodio)
nl-NL	Dutch (Netherlands)	isovalerylglycine:mol/volume:moment:liquor cerebrospinalis:kwantitatief:
pl-PL	Polish (Poland)	Izowalerianoglicyna:stężenie molowe:punkt w czasie:płyn mózgowo-rdzeniowy:ilościowy:
pt-BR	Portuguese (Brazil)	Isovalerilglicina:#N/A:Pt:LCR:Qn: Synonyms: ; Isovalerylgly; Substance concentration; Level; Point in time; Random; Cerebral spinal fluid; Spinal Fluid; Cerebrospinal Fl; Spinal Flu; Spinal Fld; Quantitative; QNT; Quant; Quan; Chemistry
ru-RU	Russian (Russian Federation)	Изовалерилглицин:МолКонц:ТчкВрм:ЦСЖ:Колич: Synonyms: Количественный Молярность;Молярная объёмная концентрация Точка во времени;Момент Цереброспинальная жидкость
tr-TR	Turkish (Turkey)	İzovalerilglisin:SubKons:Zmlı:BOS:Kant:
zh-CN	Chinese (China)	异戊酰甘氨酸:物质的量浓度:时间点:脑脊液:定量型: Synonyms: CSF CSF;脊髓液;Cerebral spinal fluid;脑脊髓液克分子浓度;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;瞬间脊液脊髓液脑脊液（Cerebral spinal fluid，CSF）脑脊髓液

Example Units

Unit	Source
umol/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.