PDHX Protein (Transcript Variant 1) (Myc-DYKDDDDK Tag)
Aperçu rapide pour PDHX Protein (Transcript Variant 1) (Myc-DYKDDDDK Tag) (ABIN2728549)
Antigène
Voir toutes PDHX ProtéinesType de proteíne
Origine
Source
Application
Pureté
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Attributs du protein
- Transcript Variant 1
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Purification/Conjugué
- Cette PDHX protéine est marqué à la Myc-DYKDDDDK Tag.
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Attributs du produit
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- Recombinant human PDHX / PDX1 (transcript variant 1) protein expressed in HEK293 cells.
- Produced with end-sequenced ORF clone
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Indications d'application
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Recombinant human proteins can be used for:
Native antigens for optimized antibody production
Positive controls in ELISA and other antibody assays -
Commentaires
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The tag is located at the C-terminal.
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Restrictions
- For Research Use only
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Concentration
- 50 μg/mL
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Buffer
- 25 mM Tris.HCl, pH 7.3, 100 mM glycine, 10 % glycerol.
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Stock
- -80 °C
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Stockage commentaire
- Store at -80°C. Thaw on ice, aliquot to individual single-use tubes, and then re-freeze immediately. Only 2-3 freeze thaw cycles are recommended.
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- PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
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Autre désignation
- Pdhx,pdx1
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Sujet
- 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].
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Poids moléculaire
- 53.9 kDa
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NCBI Accession
- NP_003468
Antigène
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