Anti-PDHX Antibody

Rs. 45,619.00
SKU stj28509

General Information

Product name Anti-PDHX Antibody
Short Description Rabbit polyclonal to PDHX
Description 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.
Applications WB,IF
Dilution range WB 1:500 - 1:2000
IF 1:10 - 1:100
Protein Name Anti-PDHX Antibody
Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2).
Storage Instruction Store at -20℃. Avoid freeze / thaw cycles.
Type of Usage For Research Use Only (RUO).

Product Properties

Host Rabbit
Clonality Polyclonal
Reactivity
Conjugation Unconjugated
Purification Affinity purification
Isotype IgG
Formulation Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Target

Gene ID
Gene Symbol
Molecular Weight 54.1 kDa
Database Links
Alternative Names PDHXPyruvate dehydrogenase protein X component antibodymitochondrial antibodyDihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex antibodyE3-binding protein antibodyE3BP antibodyLipoyl-containing pyruvate dehydrogenase complex component X antibodyproXPDHX antibodyPDX1
Function Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes, This specific binding is essential for a functional PDH complex
Cellular Localization Mitochondrion matrix
Tissue Specificity
Swiss-Prot Key

 

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