A portion of amino acids 41-70 from the human protein were used as the immunogen for the BCKDHB antibody.
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.
Western blot: 1:500-1:2000,Immunohistochemistry (FFPE): 1:25,Immunofluorescence: 1:25,Flow cytometry: 1:25 (1x10e6 cells)
Application Notes:
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.
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