beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+

Stable Identifier
R-HSA-70885
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+
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Mitochondrial 3-hydroxyisobutyrate dehydrogenase (HIBADH) catalyzes the reversible reaction of beta-hydroxyisobutyrate and NAD+ to form methylmalonyl semialdehyde and NADH + H+. The biochemical properties of human HIBADH are inferred from those of its better-studied porcine homologue (Robinson and Coon 1957). Unpublished crystallographic studies (PDB 2GF2) have shown the active enzyme to be a tetramer of HIBADH polypeptides whose aminoterminal 40 residues, a mitochondrial targeting sequence, have been removed.
Literature References
PubMed ID Title Journal Year
13416257 Purification and properties of beta-hydroxyisobutyric dehydrogenase

Coon, MJ, Robinson, WG

J Biol Chem 1957
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Catalyst Activity

3-hydroxyisobutyrate dehydrogenase activity of HIBADH tetramer [mitochondrial matrix]

Physical Entity
HIBADH tetramer [mitochondrial matrix] (Homo sapiens)
Activity
3-hydroxyisobutyrate dehydrogenase activity (GO:0008442)
Orthologous Events
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Bos taurus)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Caenorhabditis elegans)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Canis familiaris)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Danio rerio)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Dictyostelium discoideum)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Drosophila melanogaster)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Gallus gallus)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Mus musculus)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Rattus norvegicus)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Sus scrofa)
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Xenopus tropicalis)
Cross References
Rhea
Reviewed
Jassal, B  (2010-02-27)
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