BCOADC-E2 Human

The 2-Oxo-Acid Dehydrogenase Complex E2 (BCOADC-E2) is a crucial component of the branched-chain 2-oxo acid dehydrogenase complex (BCOADC). This complex is a mitochondrial multienzyme assembly that plays a significant role in maintaining the cellular redox state. The BCOADC-E2 is particularly notable for its involvement in the metabolism of branched-chain amino acids (BCAAs) such as leucine, isoleucine, and valine .

BCOADC-E2 is the central E2 component of the BCOADC complex. It is a recombinant protein expressed in insect Sf9 cells and has a molecular weight of approximately 47,321 Daltons . The protein is fused to a hexa-histidine purification tag, which facilitates its purification and subsequent use in various biochemical assays .

The primary function of BCOADC-E2 is to catalyze the oxidative decarboxylation of branched-chain 2-oxo acids, a critical step in the catabolism of BCAAs. This reaction is essential for energy production and the regulation of metabolic pathways .

BCOADC-E2 has significant clinical implications, particularly in the context of autoimmune diseases. Patients with primary biliary cirrhosis (PBC) often exhibit autoantibodies against BCOADC-E2 . These autoantibodies can be detected using various immunological assays, making BCOADC-E2 a valuable biomarker for diagnosing and monitoring PBC .

The recombinant BCOADC-E2 protein is widely used in research and diagnostic applications. It is employed in standard ELISA tests, line/dot assays, and microarray assays to detect autoantibodies in patient sera . Additionally, it is used in Western blotting and other immunological techniques to study the protein’s interactions and functions .

BCOADC-E2 is typically supplied in a liquid formulation containing 20mM Tris, 300mM NaCl, and 10% glycerol at pH 8.0 . It should be stored at -80°C to maintain its stability and avoid repeated freeze-thaw cycles . For short-term use, it can be stored at 4°C for up to 2-4 weeks .