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Description

2-hydroxy-3-methylvaleric acid (HMVA) is an acid generated by L-isoleucine oxidation pathway and involves four stereoisomers (2S,3S-HMVA, 2R,3R-HMVA, 2S,3R-HMVA and 2R,3S-HMVA). Increased levels of HMVA are found in patients with maple syrup urine disease (MSUD), an inherited metabolic disease characterized by sweet-smelling urine, ketoacidosis, seizures, and neurological dysfunction. Source: Regenstrief LOINC

Basic Part Properties

Part Name
2-Hydroxy-3-Methylvalerate
Part Display Name
2-Hydroxy-3-Methylvalerate
Part Type
Component (Describes the core component or analyte measured)
Created On
2000-05-04
Construct for LOINC Short Name
2OH3Me-valerate

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

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=LP18400-9
ConceptMap translate
https://fhir.loinc.org/ConceptMap/$translate?system=http://loinc.org&code=LP18400-9

Language Variants Get Info

TagLanguageTranslation
zh-CNChinese (China)2-羟基-3-甲基戊酸
Synonyms: 2OH3Me-戊酸
fr-CAFrench (Canada)2-hydroxy-3-méthylvalérate
et-EEEstonian (Estonia)2-hüdroksü-3-metüülvaleraat
es-ESSpanish (Spain)2-Hidroxi-3-Metilvalerato
it-ITItalian (Italy)2-Idrossi-3-Metilvalerato
tr-TRTurkish (Turkey)2-Hidroksi-3-Metilvalerat
ru-RURussian (Russian Federation)2-Гидрокси-3-Метилвалерат
nl-NLDutch (Netherlands)2-hydroxy-3-methylvaleraat
fr-BEFrench (Belgium)2-hydroxy-3-méthylvalérate
fr-FRFrench (France)2-hydroxy-3-méthylvalérate
pl-PLPolish (Poland)2-Hydroksy-3-Metylowalerian
Synonyms: Kwas 2-hydroksy-3-metylobutanowy
el-GRGreek (Greece)2-υδροξυ-3-μεθυλοβαλεριανικό
Synonyms: 2-υδροξυ-3-μεθυλοβαλεριανικό