The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.

ODBA

2-oxoisovalerate dehydrogenase (lipoamide); 2-oxoisovalerate dehydrogenase subunit alpha; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1-alpha; BCKDHA; branched chain keto acid dehydrogenase E1 alpha protein; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; mitochondrial;  BCKDE1A; BCKDHA; MSU; MSUD1; OVD1A

P12694

50 kDa

This Abmart monoclonal antibody is produced by immunizing mice with a sythetic peptide(KLH coupled)corresponding to amino-terminal residues of human ODBA

Mouse IgG

Human,Mouse,Rat

WB, IP, FACS

Western blotting        1:1000-1:5000                    
Immunoprecipitation     10 tests                
FACS                    1:100-1:200

Store at -20°C, stable for one year from the date of shipment.