ODPX Rabbit Polyclonal Antibody

SKU: BT-AP11651

Size:100μL

Background:The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits| E1| E2| and E3| two regulatory subunits| E1 kinase and E1 phosphatase| and a non-catalytic subunit| E3 binding protein (E3BP). This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor 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 eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

Research_area:Cancer; Metabolism; Signal transduction

Target_protein:PDHX

applications:WB

Reactivity:Human, Mouse

Clonality:Polyclonal

Clone ID:

Host:Rabbit

Isotype:IgG

Gene Symbol/ Name:ODPX

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):55110

UniProt accession:Human: O00330; Mouse: Q8BKZ9

Synonyms:ODPX

GeneID:Human: 8050; Mouse: 27402