Background:

Primary biliary cirrhosis (PBC) is a chronic, destructive autoimmune liver disease characterized by the presence of antimitochondrial autoantibodies in patient’s serum and T cell-mediated destruction of the biliary epithelial cells lining the small intrahepatic bile ducts. Patient sera are characterized by a high frequency (greater than 95%) of autoantibodies directed to a mitochondrial antigen, identified as the E2 component of the pyruvate dehydrogenase multienzyme complex (PDC-E2). PDC-E2 contains both an amino-terminal lipoyl-bearing domain and a carboxy-terminal catalytic domain. The human sequence preserves the Glu-Thr-Asp-Lys-Ala motif of the lipoyl-bearing site. Two conformationally alternative forms of the PDC-E2 protein have been revealed by immunoblotting. The immunodominant autoepitopes of the autoantigens correspond to the inner lipoyl domain. A significant number of asymptomatic patients found to have antibodies to PDC-E2 are at high risk of developing primary biliary cirrhosis.

Specificity:

DLAT polyclonal antibody detects endogenous levels of DLAT protein.

Alternative names:

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; DLAT; DLTA

Species reactivity:

Human,Mouse,Rat

Host:

Rabbit

Ig type:

Rabbit IgG, 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 rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Molecular weight:

~ 69 kDa

Applications:

WB IF

Usage:

WB: 1:500~1:2000 IF: 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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