Conjugation: HRP: Horseradish Peroxidase
Description of Target: 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.
Immunogen: The immunogen is a synthetic peptide directed towards the N-terminal region of Human BCKDHB
Molecular Weight: 23kDa
Peptide Sequence: Synthetic peptide located within the following region:
GFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNProduct Format: Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.
Protein Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial Ensembl ENSP00000443564 Ensembl ENSP00000358775
Protein Size: 218
Purification: Affinity Purified
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