ARP8050-01 [Polyclonal Antibody]
PDHX Rabbit Polyclonal Antibody
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Species:   Rabbit
Applications:   WB IHC
Immunogen Range:   A recombinant protein of human PDHX
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:   8050
Swiss Prot:   O00330
Synonyms:   E3BP, OPDX, PDX1, proX, DLDBP
Purification:   Affinity purification
Storage:   Store at -20oC (regular) or -80oC (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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.
Caculated MW:   54kDa
Observed MW:   Refer to Figures
Applications:   WB 1:500 - 1:2000
IHC 1:50 - 1:200
Reacitivity:   Human
For research use only. Not intended for diagnostic or therapeutic use!
Additional information