Pyruvate Dehydrogenase E2 Recombinant Rabbit mAb
Cat No.: ARM2154
Size：
| Product Name: | Pyruvate Dehydrogenase E2 Recombinant Rabbit mAb |
| Cat No.: | ARM2154 |
| source: | Rabbit |
| reactivity: | Human, Mouse, Rat |
| applications: | WB,ICC/IF,FC,IP |
| clonality: | Monoclonal |
| recommended dilution: | WB,ICC/IF,FC,IP |
| format: | Liquid |
| isotype: | IgG |
| immunogen: | A synthetic peptide of human Pyruvate Dehydrogenase E2 |
| calculated molecular weight: | 69 kDa |
| observed molecular weight: | 69 kDa |
| genbank accession number: | P10515 |
| gene id (ncbi): | 1737 |
| purification method: | Affinity Purification |
| conjugate: | Un-conjugated |
| storage: | Store at -20°C. Supplied in 50nM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt. |
| synonyms: | E2; PBC; DLTA; PDCE2; PDC-E2 |
| category: | Primary Ab |
| concentration: | 1mg/ml |
| background: | 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.[provided by RefSeq, Oct 2009] |