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.

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Immunocytochemical staining of HeLa cells using Pyruvate Dehydrogenase E2 antibody , Top panel: wild-type (WT); Bottom panal: Pyruvate Dehydrogenase E2 shRNA knockdown (KD). Nuclei were stained blue with DAPI; Acyl-CoA dehydrogenase short chain was stained magenta with Alexa Fluor® 647. Scale bar, 20 μm.

Flow cytometric analysis of Pyruvate Dehydrogenase E2 expression in HepG2 cells using Pyruvate Dehydrogenase E2 antibody . Green, isotype control; red, Pyruvate Dehydrogenase E2.

Immunocytochemical staining of HepG2 cells with Pyruvate Dehydrogenase E2 antibody . Nuclei were stained blue with DAPI; Pyruvate Dehydrogenase E2 was stained magenta with Alexa Fluor® 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Low. Scale bar, 20 μm.

Western blotting analysis using Pyruvate Dehydrogenase E2 antibody . Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with Pyruvate Dehydrogenase E2 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively. Image was developed using FeQ™ ECL Substrate Kit .

Western blotting analysis using Pyruvate Dehydrogenase E2 antibody . Pyruvate Dehydrogenase E2 expression in wild type (WT) and Pyruvate Dehydrogenase E2 shRNA knockdown (KD) HT-1080 cells with 30 μg of total cell lysates. β-Tubulin serves as a loading control. The blot was incubated with Pyruvate Dehydrogenase E2 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively. Image was developed using FeQ™ ECL Substrate Kit .

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