APPLICATION
Application Note
*Optimal dilutions/concentrations should be determined by the researcher.
Application |
Recommended Dilution |
0.1-1μg/ml |
Not tested in other applications.
Calculated MW
Positive Control
The peptide used to generate this antibody is available for purchase (GTX89576-PEP).
Product Note
This antibody is expected to recognise all reported isoforms.
Predict Reactivity
Mouse, Rat, Dog(>80% identity)
PROPERTIES
Form
Liquid
Buffer
TBS, 0.5% BSA
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
0.50 mg/ml (Please refer to the vial label for the specific concentration.)
Antigen Species
Human
Immunogen
Peptide with sequence C-KSFKANLENPIRLA, from the C Terminus of the protein sequence according to NP_003468.2; NP_001128496.1; NP_001159630.1.
Purification
Purified by ammonium sulphate precipitation followed by antigen affinity chromatography
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 , PDHXD , 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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GTX89576 WB Image
WB analysis of human pancreas lysate using GTX89576 PDHX antibody, C-term. Dilution : 1μg/ml Loading : 35μg protein in RIPA buffer
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REFERENCE
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REVIEW
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