BCKDHA Protein

Description:
|Introduction The BCKD (branched-chain alpha-keto acid dehydrogenase) complex is an inner mitochondrial enzyme complex which catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. This complex consists of 3 catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). Mutations in the BCKDHA gene result in maple syrup urine disease, type IA. |Reco|E. coli-derived. Predicted molecular mass: 47.8 kDa. Purity > 80%, by SDS-|The BCKDHA solution (0.25 mg/ml) contains 20 mM Tris-HCl buffer (pH 8.0), 5 mM DTT, 30% glycerol and 0.2 M NaCl. Shipping: The product is shipped at a|Use a manual defrost freezer and avoid repeated freeze-thaw cycles. 12 months from date of receipt, -20 to -70°C as supplied. 1 month, 2 to 8°C under sterile conditions after reconstitution. 3 months, -20 to -70°C under sterile conditions after reconstitution.