3-hydroxyisobutyrate dehydrogenase

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3-hydroxyisobutyrate dehydrogenase
Identifiers
ExPASy
NiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
Search
PMCarticles
PubMedarticles
NCBIproteins
HIBADH
Gene ontology
Molecular function
Cellular component
Biological process
Sources:Amigo / QuickGO
Ensembl
UniProt
RefSeq (mRNA)

NM_152740

NM_145567

RefSeq (protein)

NP_689953

NP_663542

Location (UCSC)Chr 7: 27.53 – 27.66 MbChr 6: 52.52 – 52.62 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

2-methyl-3-oxopropanoate, NADH, and H+
.

This enzyme belongs to the family of

valine, leucine and isoleucine degradation
.

Function

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

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

  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:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. PMID 13416257
    .
  6. ^ a b "Entrez Gene: HIBADH 3-hydroxyisobutyrate dehydrogenase".

Further reading

External links

  • 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