3-hydroxyisobutyrate dehydrogenase

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3-hydroxyisobutyrate dehydrogenase
Identifiers
EC number1.1.1.31
CAS number9028-39-1
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
HIBADH
Protein HIBADH PDB 2gf2.png
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesHIBADH, Hibadh, 6430402H10Rik, AI265272, NS5ATP1, 3-hydroxyisobutyrate dehydrogenase
External IDsMGI: 1889802 HomoloGene: 15088 GeneCards: HIBADH
EC number1.1.1.31
Gene location (Human)
Chromosome 7 (human)
Chr.Chromosome 7 (human)[1]
Chromosome 7 (human)
Genomic location for HIBADH
Genomic location for HIBADH
Band7p15.2Start27,525,442 bp[1]
End27,662,883 bp[1]
RNA expression pattern
PBB GE HIBADH gnf1h05976 at fs.png
More reference expression data
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_152740

NM_145567

RefSeq (protein)

NP_689953

NP_663542

Location (UCSC)Chr 7: 27.53 – 27.66 MbChr 6: 52.55 – 52.64 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

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[5] that in humans is encoded by the HIBADH gene.[6]

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.

Function[edit]

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.[6]

Structural studies[edit]

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

References[edit]

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000106049 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000029776 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:".
  4. ^ "Mouse PubMed Reference:".
  5. ^ Robinson WG, Coon MJ (March 1957). "The purification and properties of beta-hydroxyisobutyric dehydrogenase". J. Biol. Chem. 225 (1): 511–21. PMID 13416257.
  6. ^ a b "Entrez Gene: HIBADH 3-hydroxyisobutyrate dehydrogenase".

Further reading[edit]

External links[edit]

  • Human HIBADH genome location and HIBADH gene details page in the UCSC Genome Browser.
  • PDBe-KB provides an overview of all the structure information available in the PDB for Human 3-hydroxyisobutyrate dehydrogenase, mitochondrial