Product Description

PDHX Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS8207-50, BS8207-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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 regμlatory 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 resμlts 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 resμlts in mμltiple transcript variants encoding distinct isoforms.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IP

Alternate Names: PDHX, DLDBP, E3BP, OPDX, PDX1, proX

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

Immunogen: Recombinant fusion protein of human PDHX(NP_003468.2).

Dilution: WB, 1:500 - 1:2000 IP, 1:50 - 1:200

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).

Specificity: Polyclonal Antibodies

Molecular Weight: 54kDa

Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

SwissProt: O00330

Product: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Research Use Only