PDHX Antikörper (Middle Region)

Produktnummer ABIN5515028

Produktdetails anti-PDHX Antikörper

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

Bindungsspezifität: Middle Region

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser PDHX Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Sequenz: NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP

Aufreinigung: Affinity purified

Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human PDHX

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

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: For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent 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.

Alias Symbols: E3BP, OPDX, PDX1, proX, DLDBP

Protein Size: 501

Gen-ID: 8050

NCBI Accession: NM_001135024, NP_001128496

UniProt: O00330