DLAT (Dihydrolipoyllysine-residue Acetyltransferase Component of Pyruvate Dehydrogenase Complex, Mitochondrial, 70kD Mitochondrial Autoantigen of Primary Biliary Cirrhosis, PBC, Dihydrolipoamide Acetyltransferase, Component of Pyruvate Dehydrogenase Complex)

Size

£479.00

SKU:: 360108
Purity:: Purified by immunoaffinity chromatography
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Full-length recombinant protein corresponding to human DLAT
Format:: Supplied as a liquid in PBS, pH 7.2, 0.02% sodium azide, 50% glycerol
Extra Details:: 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: 1:500-1:2000 Immunofluorescence: 1:50-1:200 Optimal 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.

Shipping Conditions:: Blue Ice