Biology:3-hydroxyisobutyrate dehydrogenase

Short description: Protein-coding gene in the species Homo sapiens

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 / QuickGO
Search PMC articles PubMed articles NCBI proteins

Generic protein structure example

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⁺ [math]\displaystyle{ \rightleftharpoons }[/math] 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.

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.

Further reading

• "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).". Genome Res. 14 (10B): 2121–7. 2004. doi:10.1101/gr.2596504. PMID 15489334. 
• "The DNA sequence of human chromosome 7.". Nature 424 (6945): 157–64. 2003. doi:10.1038/nature01782. PMID 12853948. Bibcode: 2003Natur.424..157H. 
• "Human chromosome 7: DNA sequence and biology.". Science 300 (5620): 767–72. 2003. doi:10.1126/science.1083423. PMID 12690205. Bibcode: 2003Sci...300..767S. 
• 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. PMID 12477932. Bibcode: 2002PNAS...9916899M. 
• Sanger Centre, The; Washington University Genome Sequencing Cente, The (1999). "Toward a complete human genome sequence.". Genome Res. 8 (11): 1097–108. doi:10.1101/gr.8.11.1097. PMID 9847074. 
• "Human liver protein map: update 1993.". Electrophoresis 14 (11): 1216–22. 1994. doi:10.1002/elps.11501401181. PMID 8313870. 
• "Purification and characterization of 3-hydroxyisobutyrate dehydrogenase from rabbit liver.". J. Biol. Chem. 263 (1): 327–31. 1988. doi:10.1016/S0021-9258(19)57396-3. PMID 3335502. 
• "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. 1989. doi:10.1016/S0021-9258(18)83634-1. PMID 2647728. 

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