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GBE1 Antikörper (AA 101-200) (Cy7)

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch GBE1 in WB, IF (cc) und IF (p). Er zeigt eine Reaktivität gegenüber Human.
Produktnummer ABIN1707697

Kurzübersicht für GBE1 Antikörper (AA 101-200) (Cy7) (ABIN1707697)

Target

Alle GBE1 Antikörper anzeigen
GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

Reaktivität

  • 50
  • 12
  • 4
  • 1
  • 1
  • 1
  • 1
  • 1
Human

Wirt

  • 41
  • 9
Kaninchen

Klonalität

  • 44
  • 6
Polyklonal

Konjugat

  • 23
  • 3
  • 3
  • 3
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
Dieser GBE1 Antikörper ist konjugiert mit Cy7

Applikation

  • 41
  • 18
  • 12
  • 12
  • 12
  • 7
  • 6
  • 5
  • 4
  • 4
  • 3
  • 1
Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
  • Bindungsspezifität

    • 14
    • 7
    • 6
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 101-200

    Homologie

    Human,Mouse,Rat,Rabbit

    Aufreinigung

    Purified by Protein A.

    Immunogen

    KLH conjugated synthetic peptide derived from human GBE1

    Isotyp

    IgG
  • Applikationshinweise

    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200

    Beschränkungen

    Nur für Forschungszwecke einsetzbar
  • Format

    Liquid

    Konzentration

    1 μg/μL

    Buffer

    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

    Konservierungsmittel

    ProClin

    Vorsichtsmaßnahmen

    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

    Lagerung

    -20 °C

    Informationen zur Lagerung

    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

    Haltbarkeit

    12 months
  • Target

    GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

    Andere Bezeichnung

    GBE1

    Hintergrund

    Synonyms: 1,4 alpha glucan branching enzyme, 4-alpha-glucan-branching enzyme, amylo 1,4 to 1,6 transglucosidase, amylo 1,4 to 1,6 transglycosylase, Andersen disease, Brancher enzyme, GBE 1, GBE, GBE1, gGlucan 1,4 alpha , branching enzyme 1, GLGB_HUMAN, Glucan 1,4 alpha branching enzyme, Glycogen branching enzyme, Glycogen storage disease type IV, Glycogen-branching enzyme, OTTHUMP00000213788, OTTHUMP00000213833.

    Background: GBE1 is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy.

    Gen-ID

    2632

    Pathways

    Cellular Glucan Metabolic Process
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