3-hydroxyisobutyrate dehydrogenase
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In enzymology, a 3-hydroxyisobutyrate dehydrogenase (Template:EC number) 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+ 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.[2]
Structural studies
As of late 2007, five structures have been solved for this class of enzymes, with PDB accession codes Template:PDB link, Template:PDB link, Template:PDB link, Template:PDB link, and Template:PDB link.
References
Further reading
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External links
- Template:UCSC gene info
- PDBe-KB provides an overview of all the structure information available in the PDB for Human 3-hydroxyisobutyrate dehydrogenase, mitochondrial
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