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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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