Aliases for HIBADH Gene
External Ids for HIBADH Gene
Previous GeneCards Identifiers for HIBADH Gene
This gene encodes a mitochondrial 3-hydroxyisobutyrate dehydrogenase enzyme. The encoded protein plays a critical role in the catabolism of L-valine by catalyzing the oxidation of 3-hydroxyisobutyrate to methylmalonate semialdehyde. [provided by RefSeq, Nov 2011]
GeneCards Summary for HIBADH Gene
HIBADH (3-Hydroxyisobutyrate Dehydrogenase) is a Protein Coding gene. Diseases associated with HIBADH include 3-Hydroxyisobutyric Aciduria and Maple Syrup Urine Disease. Among its related pathways are Valine, leucine and isoleucine degradation and Metabolism. Gene Ontology (GO) annotations related to this gene include NAD binding and 3-hydroxyisobutyrate dehydrogenase activity. An important paralog of this gene is GLYR1.