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PDHX antibody

Catalog Number:OM267952
OM267952
Discount Price
OM267952-100ul
1~2 Week
100ul
OM267952-50ul
50ul
OM267952-20ul
20ul
1~2 Week
Product Profile
Product Name PDHX antibody
Antibody Type Primary Antibodies
Product description Rabbit Polyclonal antibody to PDHXThe 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
Immunogen Recombinant protein of human PDHX
Key Feature
Clonality Polyclonal
Isotype IgG
Host Species Rabbit
Tested Applications ICC/IFIHC-PWB
WB: 1:500 - 1:2000. IHC: 1:50 - 1:200:
Species Reactivity Human
Concentration 1mg/ml
Purification Affinity purified
Target Information
Gene Synonyms DLDBP
E3BP
proX
OPDX
PDX1
pyruvate dehydrogenase complex
component X
Target PDHX
Molecular Weight(MW) 54 kDa (note)
Application

Application

WB analysis of extracts from various cells using PDHX antibody.

Application

ICC/IF analysis of U2OS cells using PDHX antibody (red) and DAPI (blue).
Application Notes WB: 1:500 - 1:2000. IHC: 1:50 - 1:200:
Additional Information
Form Liquid
Storage Instructions Store at -20°C (regular) or -80°C (long term). Avoid freeze / thaw cycles.
Storage Buffer PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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