Application Note: WB: 0.1-1μg/ml. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
Calculated MW: 54
Positive Control: The peptide used to generate this antibody is available for purchase (GTX89576-PEP).
Specificity/Sensitivity: This antibody is expected to recognise all reported isoforms.
Form: Liquid
Buffer (with preservative): TBS, 0.5% BSA, 0.02% Sodium azide.
Concentration: 0.50 mg/ml (Please refer to the vial label for the specific concentration.)
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.[provided by RefSeq, Oct 2009]
Uniprot ID: O00330
Antigen Species: Human
Immunogen: Peptide with sequence C-KSFKANLENPIRLA, from the C Terminus of the protein sequence according to NP_003468.2; NP_001128496.1; NP_001159630.1.
Purification: Purified by ammonium sulphate precipitation followed by antigen affinity chromatography
Conjugation: Unconjugated
Full Name: pyruvate dehydrogenase complex component X
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