3-hydroxyisobutyrate dehydrogenase
Protein-coding gene in the species Homo sapiens
From Wikipedia, the free encyclopedia
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 | |||||||||
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| EC no. | 1.1.1.31 | ||||||||
| CAS no. | 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 | ||||||||
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External IDsOMIM: 608475; MGI: 1889802; HomoloGene: 15088; GeneCards: HIBADH; OMA:HIBADH - orthologs
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| Identifiers | |||||||||||||||||||||||||||||||||||||||||||||||||||
| Aliases | HIBADH, Hibadh, 6430402H10Rik, AI265272, NS5ATP1, 3-hydroxyisobutyrate dehydrogenase | ||||||||||||||||||||||||||||||||||||||||||||||||||
| External IDs | OMIM: 608475; MGI: 1889802; HomoloGene: 15088; GeneCards: HIBADH; OMA:HIBADH - orthologs | ||||||||||||||||||||||||||||||||||||||||||||||||||
| EC number | 1.1.1.31 | ||||||||||||||||||||||||||||||||||||||||||||||||||
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3-Hydroxyisobutyrate dehydrogenase catalyzes the chemical reaction:
The two substrates of this enzyme are 3-hydroxyisobutyric acid and oxidised nicotinamide adenine dinucleotide (NAD+). Its products are methylmalonic acid semialdehyde, reduced NADH, and a proton.[7]
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.[6]
