Recombinant Human DBT fused with hexa-histidine purification tag was expressed in Insect cells.
The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.
DLST (0.91mg/ml) is supplied in 20mM HEPES buffer pH-8.0, 200mM NaCl and 20% glycerol.
Greater than 75% as determined by SDS-PAGE.
Western blot with anti-M2-Antigen autoantibody-positive patient sera or monoclonal anti-hexa-His-tag antibody.Coating concentration; 0.4-0.8 µg/ml (depending on the type of ELISA plate and coating buffer). Suitable for biotinylation and iodination.Standard ELISA test (checkerboard analysis of positive/negative sera panels); immunodot test with positive/negative sera panels.0.4-0.8 µg/ml (depending on the type of ELISA plate and coating buffer). Suitable for biotinylation and iodination.
Store at 4 centigradeif entire vial will be used within 2-4 weeks. Store, frozen at -20 centigradefor longer periods of time.Avoid multiple freeze-thaw cycles.