Rabbit monoclonal antibody raised against a human BCKDHB peptide using ARM Technology.
A synthetic peptide of human BCKDHB is used for rabbit immunization. Customer or Abnova will decide on the preferred peptide sequence.
Quality Control Testing:
Antibody reactive against human BCKDHB peptide by ELISA and mammalian transfected lysate by Western Blot.
In 1x PBS, pH 7.4
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
1. Customer may provide cell or tissue lysate for antibody screening. 2. Rabbit monoclonal antibody generated by ARM technology is amenable to antibody engineering including F(ab)2, IgG, scFv and different Fc and non-Fc conjugates per customer request.
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. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform. [provided by RefSeq