Product Description

PDC-E2 Monoclonal Antibody

Size: 50μL, 100μL

Catalogue Numbers: RA11825-50, RA11825-100

Citations, Manuals and MSDS Available upon request.

Background: dihydrolipoamide S-acetyltransferase (DLAT) Homo sapiens 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 lact

Condition: Purified mouse monoclonal in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.

Storage Instructions: -20°C/1 year

Applications: WB

Species Cross-Reactivity: Human; Rabbit

GeneID (Human): 1737

SWISS: P10515

Source: Mouse

Research Use Only