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You are here:Home » Antibodies » Abs to Protein Kinase Substrates » Anti -Pyruvate dehydrogenase (PDHX, E3BP, OPDX, PDX1, proX, DLDBP, pyruvate dehydrogenase complex, component X, E3-binding protein, pyruvate dehydrogenase complex, lipoyl-containing component X)

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

Pricing

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Specifications

Clone Host Grade Applications
Polyclonal Goat Affinity Purified E B IH
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.
Catalog #P9544-01
ApplicationsSuitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested.
Recommended DilutionELISA (Peptide): 1:32,000
Western Blot: 0.1-1ug/ml ~50kDa band observed in Human Pancreas lysate (calculated MW of 54.2kDa according to NP_003468.1). In transfected HEK293 transiently expressing PDHX a band of approx. 50kDa is observed. This band is not observed in the non-transfected HEK293.
Immunohistochemistry (Paraffin): 1-3ug/ml Shows cytoplasmic staining of acinar cells, islets of Langerhans and peri-pancreatic fat cells in human pancreas.
Optimal dilutions to be determined by the researcher.
Peptide BlockingP9544-01-P: Pyruvate dehydrogenase, corresponding peptide
Storage and StabilityMay be stored at 4°C for short-term only. For long-term storage, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Clone TypePolyclonal
IsotypeIgG
HostGoat
SourceHuman
Concentration~0.25mg/ml
FormSupplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide, before the addition of glycerol to 40%.
PurityPurified by immunoaffinity chromatography.
ImmunogenSynthetic peptide, C-KSFKANLENPIRLA from the C terminus of human Pyruvate dehydrogenase.
SpecificityRecognizes human Pyruvate dehydrogenase at ~50kD. Species Sequence Homology: mouse, rat, canine
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.


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