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.

Target	PDHX
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Tested Applications	WB, IF/ICC
Recommended dilutions	WB: 1/500 - 1/2000, IF/ICC: 1/10 - 1/100. Optimal dilutions/concentrations should be determined by the end user.
Immunogen	Recombinant protein of human PDHX.
Purification	Affinity purified.
Form	Liquid
Isotype	IgG
Conjugation	Unconjugated
Storage	Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
Molecular Weight	Calculated MW: 29 kDa/51 kDa/54 kDa Observed MW: 54 kDa
Swiss Prot	O00330
GeneID	8050
Gene Symbol	PDHX
Concentration	> 1 mg/ml
Buffer	PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
UNSPSC Code	12352203
Availability	Shipped within 5-10 working days.
Note	This product is for research use only.