abx004919

antibody from Abbexa

Targeting: PDHX DLDBP, E3BP, OPDX, PDX1, proX

Western blot (Recommended by provider)

Immunocytochemistry (Recommended by provider)

Antibody Data

Product number

abx004919

Provider

Abbexa

Product name

Pyruvate Dehydrogenase Complex Component X (PDHX) Antibody

Antibody type

Polyclonal

Description

PDHX Antibody is a Rabbit Polyclonal antibody against PDHX. 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.

Reactivity

Human, Mouse, Rat

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

100 µl

Concentration

> 1 mg/ml

Storage

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.