Anti-Pyruvate Dehydrogenase E2 (4A4) Mouse antibody

Anti-Pyruvate Dehydrogenase E2 (4A4) Mouse antibody

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• Material Safety Data Sheets (MSDS)

Pyruvate Dehydrogenase E2 (4A4) Mouse monoclonal antibody

WB, ICC/IF, IP

Human, Mouse

70 kDa mitochondrial autoantigen of primary biliary cirrhosis;anti DLAT; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Dihydrolipoamide;Dihydrolipoamide S Acetyltransferase;Dihydrolipoamide S-acetyltransferase (E2 compone antibody

IgG1

Antigen: Purified recombinant human Pyruvate Dehydrogenase E2 protein fragments expressed in E.coli.

Monoclonal

Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4 150 mM NaCl) with 0.02% sodium azide 50% glycerol

Store at 4°C short term. Store at -20°C long term. Avoid freeze / thaw cycle.

Swiss-Prot Acc.P10515.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.