PDHX Antibody - C-terminal region
Product Specifications
CAS Number
9007-83-4
Gene Name
Pyruvate dehydrogenase complex, component X
Gene Aliases
E3BP, OPDX, PDX1, proX, DLDBP, PDHXD
Gene ID
8050
Reactivity
Human
Immunogen
Carrier-protein conjugated synthetic peptide encompassing a sequence within the C-terminus region of human PDHX. The exact sequence is proprietary.
Target
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.
Clonality
Polyclonal
Type
Polyclonal Antibody
Applications
WB
Purification
Purified by antigen-affinity chromatography.
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions
Reconstitution & Storage Instructions
Western Blotting/Immunoblotting (WB/IB) Protocol
Western Blotting/Immunoblotting (WB/IB) Protocol
Immunohistochemistry (IHC) Protocol
Immunohistochemistry (IHC) Protocol
Immunocytochemistry (ICC) Protocol
Immunocytochemistry (ICC) Protocol
Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol
Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol
Blocking Peptide Competition Protocol (BPCP)
Blocking Peptide Competition Protocol (BPCP)
Immunoprecipitation (IP) Protocol
Immunoprecipitation (IP) Protocol
Antibody Array (AA) Protocol
Antibody Array (AA) Protocol
Concentration
0.75mg/ml
Format
Liquid. 1XPBS, 10% Glycerol (pH7) . 0.01% Thimerosal was added as a preservative.
Reconstitution
Keep as concentrated solution. Aliquot and store at -20oC or below. Avoid multiple freeze-thaw cycles.
Fragment
IgG
NCBI Gene Symbol
PDHX
Host or Source
Rabbit
Gene Name URL
PDHX
Frequently Asked Questions
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