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

Stable Identifier
R-HSA-508473
Type
Reaction
Species
Homo sapiens
Compartment
mitochondrial matrix
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methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+
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Mitochondrial 3-hydroxyisobutyrate dehydrogenase (HIBADH) catalyzes the reversible reaction of methylmalonyl semialdehyde and NADH + H+ to form beta-hydroxyisobutyrate and NAD+. 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 Unpublished crystallographic studies (PDB 2GF2) have shown the active enzyme to be a tetramer of HIBADH polypeptides whose aminoterminal 40 residues, a mitochondrial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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Event Information
Reverse Reaction
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (Homo sapiens)
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
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Caenorhabditis elegans)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Canis familiaris)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Danio rerio)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Dictyostelium discoideum)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Drosophila melanogaster)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Gallus gallus)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Mus musculus)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Rattus norvegicus)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Sus scrofa)
methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ (Xenopus tropicalis)
Cross References
Rhea
Authored
D'Eustachio, P  (2010-02-18)
Reviewed
Jassal, B  (2010-02-26)
Created
D'Eustachio, P  (2010-02-12)
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