PDHX Rabbit pAb
Sizes: 20μL, 100μL
Catalogue Numbers: A6426-20, A6426-100
Citations, Manuals and MSDS Available upon request.
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.
Category: Polyclonal Antibodies
Applications: WB, IP, ELISA
Cross-reactivity: Human, Mouse, Rat
Protein Weight: 54kDa
Observed Molecular Weight: 54kDa
Immunogen: Recombinant protein (or fragment) .
Species: Human
GeneID: 8050
SWISS: O00330
Alternate Names: E3BP; OPDX; PDX1; proX; DLDBP; PDHXD; PDHX
Source: Rabbit
Isotype: IgG
Purity: Affinity purification
Storage: Store at -20℃. Avoid freeze / thaw cycles. Buffer: PBS containing 50% glycerol, preserved with proclin300 or sodium azide (as specified on the Certificate of Analysis), pH 7.3.
Recommended Dilutions: WB, 1:500 - 1:2000 IP, 0.5μg-4μg antibody for 400μg-600μg extracts of whole cells ELISA, Recommended starting concentration is 1 μg/mL. Please optimize the concentration based on your specific assay requirements.
Research Areas: Cancer, Signal Transduction, Endocrine Metabolism, Carbohydrate metabolism, Warburg Effect.
NCBI Alias: PDHX
Research Use Only