DLAT(70kD Mitochondrial Autoantigen of primary Biliary Cirrhosis,Dihydrolipoamide Acetyltransferase Component of Pyruvate Dehydrogenase Complex,Dihydrolipoamide S Acetyltransferase,Dihydrolipoyllysine-residue Acetyl

Antibodies / Antibodies-Enzymes, Dehydrogenase

-20°C

Supplied as a liquid 0.1M Tris-Glycine, pH 7.4, 150mM NaCl, 0.2% sodium azide, 50% glycerol

Recombinant full length Human DLAT.

This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.

Applications:
Suitable for use in Western Blot, Immunoprecipitation, Immunocytochemistry. Other applications not tested.

Recommended Dilution:
Western Blot: 1:1000
Immunocytochemistry: 1:300
Immunoprecipitation: 1:50-1:200
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.