DLAT polyclonal antibody

P10515

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.

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

DLAT polyclonal antibody detects endogenous levels of DLAT protein.

ca. 69 kDa

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