Product Description

PDC-E2 Recombinant Rabbit mAb

Sizes: 50μl, 100μl

Catalogue Numbers: BS66207-50, BS66207-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: This gene encodes component E2 of the mμlti-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.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IF/ICC

Alternate Names: DLTA, Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial, 70 kDa mitochondrial autoantigen of primary biliary cirrhosis, PBC, Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex, M2 antigen complex 70 kDa subunit, Pyruvate dehydrogenase complex component E2, PDC-E2, PDCE2

Clonality: Monoclonal

Conjugate: Unconjugated

Modification: Unmodified

Immunogen: KLH-conjugated synthetic peptide encompassing a sequence within human PDC-E2 protein.

Dilution: WB: 1:500~1:1000
IF/ICC: 1:50~1:200

Purification: The antibody was affinity-purified from cell cμlture supernatant by protein A+G and the purity is > 95% (by SDS-PAGE).

Specificity: PDC-E2 Monoclonal Antibody detects endogenous levels of PDC-E2 protein.

Molecular Weight: ~ 69 kDa

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

SwissProt: P10515

Product: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Research Use Only