PDC-E2 Monoclonal Antibody

SKU: BT-MCA1008

Size:100μL

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 lact

Research_area:Signal transduction; Metabolism

Target_protein:DLAT

applications:WB

Reactivity:Human,Rabbit

Clonality:Monoclonal

Clone ID:

Host:Mouse

Isotype:IgG

Gene Symbol/ Name:Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial

Immunogen:The antiserum was produced against synthesized peptide derived from human AMPK alpha around the phosphorylation site of Thr172. AA range:140-189

Storage:-20°C for 1 year

Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Formulation:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Concentration:1 mg/ml

Molecular weight(Da):N/A

UniProt accession:Human: P10515; Mouse: Q8BMF4; Rat: P08461

Synonyms:DLAT; DLTA; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex; mitochondrial; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruva

GeneID:Human: 1737