HIBADH, Official Symbol HIBADHprovided by HGNC Official Full Name 3-hydroxyisobutyrate dehydrogenaseprovided by HGNC Primary source HGNC:HGNC:4907 See related Ensembl:ENSG00000106049 MIM:608475; AllianceGenome:HGNC:4907 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as NS5ATP1 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] Expression Ubiquitous expression in kidney (RPKM 59.3), adrenal (RPKM 50.9) and 24 other tissues See more Orthologs mouse all