Primary biliary cirrhosis (PBC) is a chronic, destructive autoimmune liver disease characterized by the presence of antimitochondrial auto antibodies 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 N-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 auto antigens 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.
Applications:Suitable for use in Western Blot and Immunofluorescence.
Other applications not tested.
Recommended Dilutions:Western Blot: 1:500-1:2000Immunofluorescence: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography
Form：Supplied as a liquid in PBS, pH 7.2, 0.02% sodium azide, 50% glycerol
Specificity：Recognizes endogenous levels of human DLAT. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：IF WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Full-length recombinant protein corresponding to human DLAT
