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

Stable Identifier
R-HSA-508473
Type
Reaction
Species
Homo sapiens
Compartment
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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 Unpublished crystallographic studies (PDB 2GF2) have shown the active enzyme to be a tetramer of HIBADH polypeptides whose aminoterminal 40 residues, a mitochondrial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
Participants
Participates
Event Information
Catalyst Activity

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

Orthologous Events
Cross References
Rhea
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Reviewed
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