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3-hydroxyisobutyrate dehydrogenase, mitochondrial (HIBADH)
Function:
- role in valine catabolism
- catalyzes the NAD-dependent, reversible oxidation of 3-hydroxybutyrate to methylmalonate
Structure: homdimer
Compartment: mitochondria
Laboratory:
- 3-hydroxyisobutyrate dehydrogenase in fibroblasts
General
dehydrogenase
mitochondrial protein
oligomerizing protein
Properties
SIZE: entity length = 336 aa
MW = 35 kD
COMPARTMENT: mitochondria
MOTIF: cofactor-binding site [40-68]
COFACTOR-BOUND: NAD
cofactor-binding site [103-104]
COFACTOR-BOUND: NAD
cofactor-binding site [108-108]
COFACTOR-BOUND: NAD
cofactor-binding site [134-134]
COFACTOR-BOUND: NAD
lysine residue {K209}
cofactor-binding site [284-284]
COFACTOR-BOUND: NAD
Database Correlations
OMIM 608475
UniProt P31937
Pfam PF03446
Entrez Gene 11112
Kegg hsa:11112
ENZYME 1.1.1.31