PDHX Antibody

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.

8050

O00330

Human, Mouse, Rat

Recombinant fusion protein containing a sequence corresponding to amino acids 1-300 of human PDHX (NP_003468.2) .

Unconjugated

Primary Antibodies

Cancer, Signal Transduction

Affinity purification

SW620

Batch dependent

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

None

Blue Ice

Store at -20˚ C. Avoid freeze / thaw cycles.

Observed: 54kDa

IgG

PDHX

HepG2

U-251MG

MCF7

Mouse heart

Mouse testis

Pyruvate dehydrogenase complex, component X

Homo sapiens

PDHX, E3BP, OPDX, PDX1, proX, DLDBP, pyruvate dehydrogenase complex, component X, E3-binding protein, lipoyl-containing component X

WB, IF