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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.
Alexa Fluor® est une marque déposée de Molecular Probes Inc., OR., USA
LI-COR® et Odyssey® sont marques déposées de LI-COR Biosciences
Informations pour la commande
Nom du produit | Ref. Catalogue | COND. | Prix HT | QTÉ | Favoris | |
Anticorps PDC-E2 (C-1) | sc-166899 | 200 µg/ml | $316.00 | |||
PDC-E2 (C-1) peptide neutralisant | sc-166899 P | 100 µg/0.5 ml | $68.00 |