PDC-E2 Recombinant Rabbit mAb

Product Specifications

Background

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.

Structure Composition

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

Product Name Alternative

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

Swiss Prot

P10515

Reactivity

Human, Mouse, Rat

Immunogen

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

Conjugation

Unconjugated

Applications

WB, IF/ICC

Dilution

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

Purity

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

Modification

Unmodified

Molecular Weight

~ 69 kDa

Storage Conditions

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

Notes

For research use only, not for use in diagnostic procedure.

Specificity

PDC-E2 mAb detects endogenous levels of PDC-E2 protein.

Applications Notes

Western blot analysis of PDC-E2 expression in K562 (A), PC3 (B), HepG2 (C), mouse kidney (D), mouse muscle (E), rat kidney (F), rat muscle (G) whole cell lysates.