The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesWB: 1/500 - 1/1000. Detects a band of approximately 48 kDa (predicted molecular weight: 50 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionThe branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Involvement in diseaseMaple syrup urine disease 1A
Sequence similaritiesBelongs to the BCKDHA family.