Branched-chain keto acid dehydrogenase is a multienzymecomplex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. Thecomplex consists of multiple copies of 3 components: branched-chainalpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2)and lipoamide dehydrogenase (E3). This gene encodes the E1 betasubunit, and mutations therein have been associated with maplesyrup urine disease (MSUD), type 1B, a disease characterized by amaple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems.
The stated application concentrations are suggested starting points. Titration of the BCKDHB antibody may be required due to differences in protocols and secondary/substrate sensitivity.
A portion of amino acids 41-70 from the human protein were used as the immunogen for the BCKDHB antibody.
Aliquot the BCKDHB antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
