HIBADH

3-hydroxyisobutyrate dehydrogenase

Summary

This gene encodes a mitochondrial 3-hydroxyisobutyrate dehydrogenase enzyme. The encoded protein plays a critical role in the catabolism of L-valine by catalyzing the oxidation of 3-hydroxyisobutyrate to methylmalonate semialdehyde. [provided by RefSeq, Nov 2011]

Known Variants25 total

rsidPosition (GRCh37)AllelesClassClinVar
rs7758284807:27,570,864C/Tuncertain significance
rs9706169177:27,570,915G/Auncertain significance
rs1404227417:27,570,942C/Alikely benign
rs14312977927:27,570,953G/Tuncertain significance
rs7651618437:27,577,997T/Cuncertain significance
rs7553075597:27,578,035G/Tuncertain significance
rs2006806487:27,582,620T/Cuncertain significance
rs2007023677:27,582,633A/Guncertain significance
rs7802134287:27,582,689G/Auncertain significance
rs126739497:27,600,634G/T
rs125397077:27,626,165T/Cintron variant
rs77904987:27,653,207A/Gintron variant
rs127008237:27,659,221G/Aintron variant
rs7752323047:27,669,040T/Cuncertain significance
rs23914487:27,678,185C/G
rs23914497:27,685,146C/Tintron variant
rs7686811887:27,689,099C/Tuncertain significance
rs3710635017:27,689,115C/Tuncertain significance
rs17864490187:27,702,319G/Auncertain significance
rs9011330477:27,702,335C/Auncertain significance
rs12578192727:27,702,337G/Auncertain significance
rs7543454337:27,702,340G/Auncertain significance
rs13999791157:27,702,377C/Tuncertain significance
rs25344720607:27,702,386G/Auncertain significance
rs125319527:27,703,830G/T

Gene information from NCBI Gene. Variant classifications from ClinVar.