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.

Specificity:

This antibody detects endogenous levels of Pyruvate Dehydrogenase E2 and does not cross-react with related proteins

Alternative names:

70 kDa mitochondrial autoantigen of primary biliary cirrhosis; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Dihydrolipoamide S Acetyltransferase; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; DLAT; DLTA; E2; E2 component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; mitochondrial; ODP2; PBC; PDC E2; PDC-E2; PDCE2; Pyruvate dehydrogenase complex component E2; Pyruvate dehydrogenase complex E2 subunit; DLTA; PDCE2; PDC-E2; Pyruvate Dehydrogenase E2

Species reactivity:

Human,Mouse

Host:

Mouse

Ig type:

Mouse IgG1, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Immunogen:

Recombinant full length Human DLAT.

Purification & Purity:

The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Molecular weight:

Predicted band size:69KDa Observed band size:69KDa

Applications:

WB ICC IP

Usage:

WB: 1:1000 ICC: 1:300 IP: 1:50~1:200

Storage:

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

Precautions:

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

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