Version 2.77

Part Description

LP18407-4   2-Oxo,3-Methylvalerate
3-Methyl-2-oxovaleric acid is a metabolite of isoleucine in man, animals and bacteria. It is the alpha-keto acid analogue of isoleucine. 3-Methyl-2-oxovaleric acid is produced from isoleucine by cytosolic branched chain aminotransferase 1 (EC:2.6.1.42), whereupon it is further degraded by branched chain keto acid dehydrogenase E1 to 2-Methyl-1-hydroxybutyl-ThPP. It is used as a clinical marker for maple syrup urine disease (MSUD). MSUD is caused by a deficiency of the branched-chain -keto acid dehydrogenase compex resulting in an accumulation of branched-chain amino acids and the corresponding -keto-and -hydroxy acids in blood, urine and cerebrospinal fluid causing neurological damage and mental retardation. 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, 2-Oxo,3-Methylvalerate

Fully-Specified Name

Component
2-Oxo,3-Methylvalerate
Property
SCnc
Time
Pt
System
Ser/Plas
Scale
Qn
Method

Additional Names

Short Name
2Oxo3Me-valerate SerPl-sCnc
Display Name
2-Oxo,3-Methylvalerate [Moles/Vol]
Consumer Name Alpha Get Info
2-Oxo,3-Methylvalerate, Blood

Basic Attributes

Class
CHEM
Type
Laboratory
First Released
Version 2.13
Last Updated
Version 2.73
Order vs. Observation
Both
Common Test Rank Get Info
9519

Member of these Panels

LOINC Long Common Name
101455-4 Mitochondrial metabolites panel - Serum or Plasma
35866-3 Organic acids panel - Serum or Plasma

Language Variants Get Info

Tag Language Translation
es-AR Spanish (Argentina) 2-oxo,3-metilvalerato:concentración de sustancia:punto en el tiempo:suero/plasma:cuantitativo:
es-ES Spanish (Spain) 2-Oxo,3-Metilvalerato:Concentración de sustancia:Punto temporal:Suero o Plasma:Qn:
Synonyms: Cuantitativo
es-MX Spanish (Mexico) 2-oxo, 3-metilvalerato:Concentración de sustancias:Punto temporal:Suero o Plasma:Cuantitativo:
fr-CA French (Canada) 2-oxo,3-méthylvalérate:Concentration de substance:Temps ponctuel:Sérum/Plasma:Quantitatif:
fr-FR French (France) 2-oxo-3-méthylvalérate:Moles/Volume:Ponctuel:Sérum/Plasma:Numérique:
fr-BE French (Belgium) 2-oxo,3-méthylvalérate:Concentration de substance:Temps ponctuel:Sérum/Plasma:Quantitatif:
it-IT Italian (Italy) 2-Osso,3-Metilvalerato:SCnc:Pt:Siero/Plasma:Qn:
Synonyms: Chimica Concentrazione di Sostanza Plasma Punto nel tempo (episodio) Siero Siero o Plasma
ko-KR Korean (Korea, Republic Of) 2-옥소,3-메틸발레레이트:물질농도:검사시점:혈청/혈장:정량:
nl-NL Dutch (Netherlands) 2-oxo,3-methylvaleraat:mol/volume:moment:serum of plasma:kwantitatief:
pt-BR Portuguese (Brazil) 2-Oxo,3-Metilvalerato:SCnc:Pt:Sor/Plas:Qn:
Synonyms: ; 2Oxo3Me-valerate; 2-keto-3-methylvaleric acid; 2-keto-3-methylvalerate; 2-oxo,3-methylvaleric acid; Substance concentration; Level; Point in time; Random; SerPl; SerPlas; SerP; Serum; SR; Plasma; Pl; Plsm; Quantitative; QNT; Quant; Quan; III; II; Chemistry
ru-RU Russian (Russian Federation) 2-Оксо,3-Метилвалерат:МолКонц:ТчкВрм:Сыв/Плаз:Колич:
Synonyms: Количественный Молярность;Молярная объёмная концентрация Плазма Сыворотка Сыворотка или Плазма Точка во времени;Момент
tr-TR Turkish (Turkey) 2-Okzo,3-Metilvalerat:SubKons:Zmlı:Ser/Plaz:Kant:
zh-CN Chinese (China) 2-氧代,3-甲基戊酸:物质的量浓度:时间点:血清/血浆:定量型:
Synonyms: 2Oxo3Me-戊酸;2-酮-3-甲基戊酸;2-酮基-3-甲基戊酸 Ⅱ Ⅲ II III 三 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 叁 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 血清或血浆

Example Units

Unit Source
mcmol/L REGENSTRIEF
umol/L 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=35869-7