This Antibody was verified by Relative expression to ensure that the antibody binds to the antigen stated. View Details
Immunogen sequence: VGKPLVDIET EALKDSEEDV VETPAVSHDE HTHQEIKGRK TLATPAVRRL AMENNIKLSE VVGSGKDGRI LKEDILNYLE KQ
Highest antigen sequence identity to the following orthologs: Mouse - 95%, Rat - 95%.
The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; BCATE2; BCKAD E2; BCKAD E2 subunit; BCKAD-E2; BCKADE2; BCOADC-E2; Branched chain 2-oxo-acid dehydrogenase complex component E2; branched chain acyltransferase; branched chain acyltransferase, E2 component; Branched-chain alpha-keto acid dehydrogenase complex component E2; branched-chain alpha-ketoacid dehydrogenase, E2 subunit; dihydrolipoamide acetyltransferase compon; Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex; Dihydrolipoamide branched chain transacylase; dihydrolipoyl transacylase; Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase; E2; E2 component; E2 component of branched chain alpha-keto acid dehydrogenase complex; E2B; Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial; lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex; mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b); part of the BCKAD complex
Gene Aliases: BCATE2; BCKAD-E2; BCKADE2; BCOADC-E2; D3Wsu60e; DBT; E2; E2B