Tel:
+49 (0)241 95 163 153
Fax:
+49 (0)241 95 163 155
E-Mail:
orders@anticorps-enligne.fr

PDHX anticorps

PDHX Reactivité: Humain, Souris, Rat IF Hôte: Lapin Polyclonal unconjugated
N° du produit ABIN7262258
  • Antigène Voir toutes PDHX Anticorps
    PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
    Reactivité
    Humain, Souris, Rat
    Hôte
    • 33
    • 3
    • 1
    • 1
    Lapin
    Clonalité
    • 37
    • 1
    Polyclonal
    Conjugué
    • 17
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp PDHX est non-conjugé
    Application
    • 30
    • 13
    • 13
    • 7
    • 6
    • 6
    • 5
    • 3
    • 3
    • 2
    • 1
    • 1
    Immunofluorescence (IF)
    Attributs du produit
    Polyclonal Antibody
    Purification
    Affinity purification
    Immunogène
    Recombinant fusion protein of human PDHX (NP_003468.2).
    Isotype
    IgG
    Top Product
    Discover our top product PDHX Anticorps primaire
  • Indications d'application
    IF 1:10-1:100
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 mg/mL
    Buffer
    PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3
    Agent conservateur
    Sodium azide
    Précaution d'utilisation
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    Stock
    -20 °C
    Stockage commentaire
    Store at -20°C. Avoid freeze / thaw cycles.
  • Antigène
    PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))
    Autre désignation
    PDHX (PDHX Produits)
    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.
    ID gène
    8050
    UniProt
    O00330
Vous êtes ici:
Support technique