Version 2.77

Part Description

LP15301-2   3-Hydroxyisovalerate
3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. Elevated levels of this compound are found in several inherited disorders such as Dihydrolipoamide dehydrogenase Deficiency, 3-Methylcrotonyl-CoA carboxylase 1 deficiency, 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (3-hydroxy-3-methylglutaryl -CoA lyase Deficiency, Biotinidase deficiency multiple carboxylase deficiency late-onset , Late onset multiple carboxylase deficiency, HolMcarboxylase synthetase deficiency, 3-Methylcrotonyl-CoA carboxylase 2 deficiency. 3-Hydroxyisovaleric acid is also elevated in smokers, in subjects undergoing long-term anticonvulsant therapy with carbamazepine and/or phenytoin. These levels are elevated due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites PMID: 16895887, PMID: 9523856, PMID: 15447901, PMID: 9176832, [OMIM: 210210], [OMIM: 253270], [OMIM: 600529], [OMIM: 253260], [OMIM: 246450], [OMIM: 210200], [OMIM: 238331] 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, Human Metabolome Database

Fully-Specified Name


Additional Names

Short Name
3OH-Isovalerate SerPl-sCnc
Display Name
3-Hydroxyisovalerate [Moles/Vol]
Consumer Name Alpha Get Info
3-Hydroxyisovalerate, Blood

Basic Attributes

First Released
Version 2.42
Last Updated
Version 2.42
Order vs. Observation

Member of these Panels

LOINC Long Common Name
101455-4 Mitochondrial metabolites panel - Serum or Plasma

Language Variants Get Info

Tag Language Translation
es-ES Spanish (Spain) 3-Hidroxiisovalerato:Concentración de sustancia:Punto temporal:Suero o Plasma:Qn:
Synonyms: Cuantitativo
es-MX Spanish (Mexico) 3-hidroxiisovalerato:Concentración de sustancias:Punto temporal:Suero o Plasma:Cuantitativo:
et-EE Estonian (Estonia) 3-hüdroksüisovaleraat:SCnc:Pt:S/P:Qn:
Synonyms: Aine kontsentratsioon Juhuslik Kvantitatiivne Plasma Seerum Seerum või plasma
fr-CA French (Canada) 3-hydroxyisovalérate:Concentration de substance:Temps ponctuel:Sérum/Plasma:Quantitatif:
fr-FR French (France) 3-hydroxyisovalérate:Moles/Volume:Ponctuel:Sérum/Plasma:Numérique:
fr-BE French (Belgium) 3-hydroxyisovalérate:Concentration de substance:Temps ponctuel:Sérum/Plasma:Quantitatif:
it-IT Italian (Italy) 3-Idrossiisovalerato:SCnc:Pt:Siero/Plasma:Qn:
Synonyms: Chimica Concentrazione di Sostanza Plasma Punto nel tempo (episodio) Siero Siero o Plasma
nl-NL Dutch (Netherlands) 3-hydroxyisovaleraat:mol/volume:moment:serum of plasma:kwantitatief:
pt-BR Portuguese (Brazil) 3-Hidroxisovalerato::Pt:Soro/Plas:Qn:
ru-RU Russian (Russian Federation) 3-Гидроксиизовалерат:МолКонц:ТчкВрм:Сыв/Плаз:Колич:
Synonyms: Количественный Молярность;Молярная объёмная концентрация Плазма Сыворотка Сыворотка или Плазма Точка во времени;Момент
tr-TR Turkish (Turkey) 3-Hidroksiizovalerat:SubKons:Zmlı:Ser/Plaz:Kant:
zh-CN Chinese (China) 3-羟基异戊酸:物质的量浓度:时间点:血清/血浆:定量型:
Synonyms: 3-HIA;3OH-异戊酸;3-羟基-异戊酸 Ⅲ 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
umol/L Example UCUM Units

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

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