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

Stable Identifier
R-HSA-70885
Type
Reaction [transition]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
(S)-3-hydroxy-2-methylpropanoate + NAD(+) => (S)-2-methyl-3-oxopropanoate + H(+) + NADH
ReviewStatus
5/5
Locations in the PathwayBrowser
Expand all
General
SVG | 
PNG
Low
Medium
High
 | PPTX  | SBGN
beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout
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 Unpublished crystallographic studies (PDB 2GF2) have shown the active enzyme to be a tetramer of HIBADH polypeptides whose aminoterminal 40 residues, a mitochondrial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
Participants
Input
Output
Participates
as an event of
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
Rhea
Reviewed
Jassal, B  (2010-02-27)
Cite Us!