3-hydroxyisobutyrate dehydrogenase

3-hydroxyisobutyrate dehydrogenase
Identifiers
EC number	1.1.1.31
CAS number	9028-39-1
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / EGO
Search PMC articles PubMed articles NCBI proteins

HIBADH
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 2GF2, 2I9P
Identifiers
Aliases	HIBADH, Hibadh, 6430402H10Rik, AI265272, NS5ATP1, 3-hydroxyisobutyrate dehydrogenase
External IDs	MGI: 1889802 HomoloGene: 15088 GeneCards: HIBADH
EC number	1.1.1.31
Gene ontology Molecular function • 3-hydroxyisobutyrate dehydrogenase activity • oxidoreductase activity • NAD binding • phosphogluconate dehydrogenase (decarboxylating) activity Cellular component • mitochondrial matrix • mitochondrion Biological process • valine catabolic process • branched-chain amino acid catabolic process • oxidation-reduction 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_689953.1	NP_663542.1
Location (UCSC)	Chr 7: 27.53 – 27.66 Mb	Chr 6: 52.55 – 52.64 Mb
PubMed search	[1]	[2]
Wikidata

View/Edit Human

View/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^[3] that in humans is encoded by the HIBADH gene.^[4]

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.

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.^[4]

Structural studies

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

References

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

External links

• Human HIBADH genome location and HIBADH gene details page in the UCSC Genome Browser.

Further reading

• Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
• Hillier LW, Fulton RS, Fulton LA, et al. (2003). "The DNA sequence of human chromosome 7.". Nature. 424 (6945): 157–64. doi:10.1038/nature01782. PMID 12853948.
• Scherer SW, Cheung J, MacDonald JR, et al. (2003). "Human chromosome 7: DNA sequence and biology.". Science. 300 (5620): 767–72. doi:10.1126/science.1083423. PMC 2882961. PMID 12690205.
• Mammalian Gene Collection Program Team; Strausberg, R. L.; Feingold, E. A.; Grouse, L. H.; et al. (2002). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proceedings of the National Academy of Sciences. 99 (26): 16899–16903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
• "Toward a complete human genome sequence.". Genome Res. 8 (11): 1097–108. 1999. doi:10.1101/gr.8.11.1097. PMID 9847074.
• Hughes GJ, Frutiger S, Paquet N, et al. (1994). "Human liver protein map: update 1993.". Electrophoresis. 14 (11): 1216–22. doi:10.1002/elps.11501401181. PMID 8313870.
• Rougraff PM, Paxton R, Kuntz MJ, et al. (1988). "Purification and characterization of 3-hydroxyisobutyrate dehydrogenase from rabbit liver.". J. Biol. Chem. 263 (1): 327–31. PMID 3335502.
• Rougraff PM, Zhang B, Kuntz MJ, et al. (1989). "Cloning and sequence analysis of a cDNA for 3-hydroxyisobutyrate dehydrogenase. Evidence for its evolutionary relationship to other pyridine nucleotide-dependent dehydrogenases.". J. Biol. Chem. 264 (10): 5899–903. PMID 2647728.