3-hydroxyisobutyrate Dehydrogenase

3-hydroxyisobutyrate Dehydrogenase

Identifiers Symbols HIBADH; NS5ATP1 External IDs OMIM: 608475 MGI: 1889802 HomoloGene: 15088 GeneCards: HIBADH Gene EC number 1.1.1.31

Gene Ontology
Molecular function	• phosphogluconate dehydrogenase (decarboxylating) activity • 3-hydroxyisobutyrate dehydrogenase activity • NAD binding
Cellular component	• mitochondrion • mitochondrial matrix
Biological process	• pentose-phosphate shunt • valine metabolic process • branched-chain amino acid catabolic process • cellular nitrogen compound metabolic process • small molecule metabolic process
Sources: Amigo / QuickGO

RNA expression pattern More reference expression data Orthologs Species Human Mouse Entrez 11112 58875 Ensembl ENSG00000106049 ENSMUSG00000029776 UniProt P31937 Q99L13 RefSeq (mRNA) NM_152740 NM_145567 RefSeq (protein) NP_689953 NP_663542 Location (UCSC) Chr 7:
27.57 – 27.7 Mb Chr 6:
52.55 – 52.64 Mb PubMed search

In enzymology, a 3-hydroxyisobutyrate dehydrogenase (EC 1.1.1.31) also known as β-hydroxyisobutyrate dehydrogenase or 3-hydroxyisobutyrate dehydrogenase, mitochondrial (HIBADH) is an enzyme that in humans is encoded by the HIBADH gene.

3-Hydroxyisobutyrate dehydrogenase catalyzes the chemical reaction:

3-hydroxy-2-methylpropanoate + NAD+ 2-methyl-3-oxopropanoate + NADH + H+

Thus, the two substrates of this enzyme are 3-hydroxy-2-methylpropanoate and NAD+, whereas its 3 products are 2-methyl-3-oxopropanoate, NADH, and H+.

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with NAD+ or NADP+ as acceptor. The systematic name of this enzyme class is 3-hydroxy-2-methylpropanoate:NAD+ oxidoreductase. This enzyme participates in valine, leucine and isoleucine degradation.