PDHX Antikörper

Produktnummer ABIN7262258

Produktdetails anti-PDHX Antikörper

Target: PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser PDHX Antikörper ist unkonjugiert

Applikation: Immunofluorescence (IF)

Produktmerkmale: Polyclonal Antibody

Aufreinigung: Affinity purification

Immunogen: Recombinant fusion protein of human PDHX (NP_003468.2).

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF 1:10-1:100

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 mg/mL

Buffer: PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Avoid freeze / thaw cycles.

Details zu PDHX

Target: PDHX (Pyruvate Dehydrogenase Complex, Component X (PDHX))

Andere Bezeichnung: PDHX (PDHX Produkte)

Synonyme: DLDBP antikoerper, E3BP antikoerper, OPDX antikoerper, PDX1 antikoerper, proX antikoerper, AI481367 antikoerper, E3bp antikoerper, Pdx1 antikoerper, Ac1164 antikoerper, zgc:66110 antikoerper, zgc:85977 antikoerper, MGC86218 antikoerper, PDHX antikoerper, DDBDRAFT_0168431 antikoerper, DDBDRAFT_0230192 antikoerper, DDB_0168431 antikoerper, DDB_0230192 antikoerper, pyruvate dehydrogenase complex component X antikoerper, pyruvate dehydrogenase complex, component X antikoerper, pyruvate dehydrogenase complex component X L homeolog antikoerper, E3-binding protein antikoerper, PDHX antikoerper, Pdhx antikoerper, pdhx antikoerper, pdhx.L antikoerper, pdhX antikoerper

Hintergrund: 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.

Gen-ID: 8050

UniProt: O00330