Product Name :BCKDHA polyclonal antibody
Application_all :WB,1:500 - 1:2000|IF/ICC,1:50 - 1:200
Background :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.
Product :1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Purification&Purity :The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity :Unmodification
Note :For research use only, not for use in diagnostic procedure.
Alternative Name :BCKDHA; BCKDE1A; MSU; MSUD1; OVD1A; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Immunogen :Recombinant fusion protein of human BCKDHA(NP_000700.1).
Modification :Unmodification
