Description

3-Hydroxyisobutyrate dehydrogenase (HIBADH) [UniProt: P31937] is a dimeric mitochondrial enzyme involved in amino-acid degradation, specifically catabolism of L-valine. Deficiency of HIBADH enzyme is thought to cause 3-hydroxyisobutyric aciduria since 3-hydroxyisobutyric acid is an intermediate in the catabolism of valine. More recent evidence suggests individuals with 3-hydroxyisobutyryic aciduria represent a heterogeneous group with different underlying mechanisms. PMID: 21863277 Source: Regenstrief LOINC

Basic Part Properties

Part Name
3-Hydroxyisobutyrate dehydrogenase
Part Display Name
3-Hydroxyisobutyrate dehydrogenase
Part Type
Component (Describes the core component or analyte measured)
Created On
2015-10-27
Construct for LOINC Short Name
HIBADH

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

CodeSystem lookup
https://fhir.loinc.org/CodeSystem/$lookup?system=http://loinc.org&code=LP201746-7

Language Variants Get Info

TagLanguageTranslation
zh-CNChinese (China)3-羟基异丁酸脱氢酶
Synonyms: 3-羟异丁酸脱氢酶
es-ESSpanish (Spain)3-Hidroxibutirato deshidrogenasa
it-ITItalian (Italy)3-idrossi isobutirrato deidrogenasi
nl-NLDutch (Netherlands)3-hydroxyisobutyraatdehydrogenase
fr-FRFrench (France)3-hydroxyisobutyrate déshydrogénase
pl-PLPolish (Poland)Dehydrogenaza 3-hydroksyizomaślanowa
el-GRGreek (Greece)3-υδροξυ-ισοβουτυρική αφυδρογονάση
Synonyms: 3-υδροξυ-ισοβουτυρική αφυδρογονάση