3-hydroxyisobutyrate dehydrogenase

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3-hydroxyisobutyrate dehydrogenase
EC number
CAS number 9028-39-1
IntEnz IntEnz view
ExPASy NiceZyme view
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum
Gene Ontology AmiGO / EGO
3-hydroxyisobutyrate dehydrogenase
Protein HIBADH PDB 2gf2.png
PDB rendering based on 2gf2.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols HIBADH ; NS5ATP1
External IDs OMIM608475 MGI1889802 HomoloGene15088 GeneCards: HIBADH Gene
EC number
RNA expression pattern
PBB GE HIBADH gnf1h05976 at tn.png
More reference expression data
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 [1] [2]

In enzymology, a 3-hydroxyisobutyrate dehydrogenase (EC also known as β-hydroxyisobutyrate dehydrogenase or 3-hydroxyisobutyrate dehydrogenase, mitochondrial (HIBADH) is an enzyme[1] that in humans is encoded by the HIBADH gene.[2]

3-Hydroxyisobutyrate dehydrogenase catalyzes the chemical reaction:

3-hydroxy-2-methylpropanoate + NAD+ \rightleftharpoons 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.


3-hydroxyisobutyrate dehydrogenase is a tetrameric mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidation of 3-hydroxyisobutyrate, an intermediate of valine catabolism, to methylmalonate semialdehyde.[2]

Structural studies[edit]

As of late 2007, 5 structures have been solved for this class of enzymes, with PDB accession codes 1WP4, 2CVZ, 2GF2, 2H78, and 2I9P.


  1. ^ Robinson WG, Coon MJ (March 1957). "The purification and properties of beta-hydroxyisobutyric dehydrogenase". J. Biol. Chem. 225 (1): 511–21. PMID 13416257. 
  2. ^ a b "Entrez Gene: HIBADH 3-hydroxyisobutyrate dehydrogenase". 

Further reading[edit]