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GBE1 antibody (AA 101-200)

GBE1 Reactivity: Human WB, ELISA, IF (cc), IF (p), ICC, IHC (p), IHC (fro) Host: Rabbit Polyclonal unconjugated
Catalog No. ABIN1713806
  • Target See all GBE1 Antibodies
    GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
    Binding Specificity
    • 14
    • 7
    • 7
    • 6
    • 6
    • 5
    • 4
    • 1
    • 1
    • 1
    • 1
    AA 101-200
    Reactivity
    • 53
    • 11
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    Human
    Host
    • 44
    • 9
    Rabbit
    Clonality
    • 47
    • 6
    Polyclonal
    Conjugate
    • 20
    • 4
    • 4
    • 4
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This GBE1 antibody is un-conjugated
    Application
    • 44
    • 20
    • 16
    • 12
    • 12
    • 6
    • 5
    • 5
    • 4
    • 3
    • 2
    • 1
    Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunocytochemistry (ICC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
    Predicted Reactivity
    Human,Mouse,Rat,Rabbit
    Purification
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human GBE1
    Isotype
    IgG
    Top Product
    Discover our top product GBE1 Primary Antibody
  • Application Notes
    WB 1:300-5000
    ELISA 1:500-1000
    IHC-P 1:200-400
    IHC-F 1:100-500
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    ICC 1:100-500
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
    Preservative
    ProClin
    Precaution of Use
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Storage
    4 °C,-20 °C
    Storage Comment
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    Expiry Date
    12 months
  • Target
    GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
    Alternative Name
    GBE1 (GBE1 Products)
    Synonyms
    glgBII antibody, APBD antibody, GBE antibody, GSD4 antibody, 2310045H19Rik antibody, 2810426P10Rik antibody, D16Ertd536e antibody, 1,4-alpha-glucan branching protein GlgB antibody, 1,4-alpha-glucan branching protein antibody, glycogen branching protein antibody, glycogen branching enzyme antibody, 1,4-alpha-glucan-branching protein antibody, glycogen branching enzyme; BE; 1,4-alpha-D-glucan:1,4-alpha-D-glucan 6-glucosyl-transferase antibody, 1,4-alpha-glucan branching enzyme 1 antibody, glucan (1,4-alpha-), branching enzyme 1 antibody, glgB antibody, ECs4277 antibody, SCO7332 antibody, Synpcc7942_1085 antibody, AZC_4116 antibody, Sros_8169 antibody, Despr_0975 antibody, GBE1 antibody, Gbe1 antibody
    Background

    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.

    Gene ID
    2632
    Pathways
    Cellular Glucan Metabolic Process
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