APPLICATION
Application Note
*Optimal dilutions/concentrations should be determined by the researcher.
Application |
Recommended Dilution |
Assay dependent |
Not tested in other applications.
Calculated MW
PROPERTIES
Form
Liquid
Buffer
PBS, 10% Glycerol
Preservative
0.02% Sodium azide
Storage
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
Concentration
1 mg/ml (Please refer to the vial label for the specific concentration.)
Antigen Species
Human
Immunogen
The clone AT1E11 is derived from hybridization of mouse F2 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human PDHX protein.
Purification
Protein A Purified
Conjugation
Unconjugated
Note
For laboratory research use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.
Purchasers shall not, and agree not to enable third parties to, analyze, copy, reverse engineer or otherwise attempt to determine the structure or sequence of the product.
TARGET
Synonyms
pyruvate dehydrogenase complex component X , DLDBP , E3BP , OPDX , PDHX , PDX1 , proX
Cellular Localization
Mitochondrion matrix
Background
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.[provided by RefSeq, Oct 2009]
Database
Research Area
DATA IMAGES
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GTX57725 WB Image
WB analysis of various samples using GTX57725 PDHX antibody. Lane 1 : A431 whole cell lysate Lane 2 : HepG2 whole cell lysate Lane 3 : 293T whole cell lysate Lane 4 : LNCap whole cell lysate Loading : 40 μg Dilution : 1:1000
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REFERENCE
There are currently no references for PDHX antibody [AT1E11] (GTX57725). Be the first to share your publications with this product.
REVIEW
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