免疫原A synthetic peptide of human Pyruvate Dehydrogenase E2
基因ID1737
蛋白编码P10515
同义词DLAT; DLTA; E2; PBC; PDCE2;
宿主物种Rabbit
抗体分型IgG
纯化Affinity purification
储存Store at -20℃. Avoid freeze / thaw cycles.
靶点背景This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.
推荐稀释比WB 1:1000-1:2000 ICC/IF 1:50-1:200 IP 1:20-1:50 FC 1:20-1:100
产品形式Liquid
Buffer 体系PBS with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
