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Anti-GBE1 Rabbit Polyclonal Antibody (Alexa Fluor® 680)
Anti-GBE1 Rabbit Polyclonal Antibody (Alexa Fluor® 680)
Catalog # BOSSBS-13300R-A680
Supplier:  Bioss
Anti-GBE1 Rabbit Polyclonal Antibody (Alexa Fluor® 680)
Catalog # BOSSBS-13300R-A680
Supplier:  Bioss

Specifications

  • Antibody type:
    Primary
  • Antigen name:
    GBE1
  • Clonality:
    Polyclonal
  • Conjugation:
    Alexa Fluor® 680
  • Host:
    Rabbit
  • ImmunoChemistry:
    Yes
  • ImmunoFluorescence:
    Yes
  • Isotype:
    IgG
  • Western blot:
    Yes
  • Epitope:
    101-200/702
  • Form:
    Liquid
  • Gene ID:
    2632
  • Antigen synonyms:
    Glycogen branching enzyme|GLGB_HUMAN|amylo 1,4 to 1,6 transglycosylase|OTTHUMP00000213788|GBE|Glycogen storage disease type IV|amylo 1,4 to 1,6 transglucosidase|Brancher enzyme|Andersen disease|Glucan 1,4 alpha branching enzyme|GBE1|GBE 1|1,4 alpha glucan branching enzyme|Glycogen-branching enzyme|OTTHUMP00000213833|4-alpha-glucan-branching enzyme|gGlucan 1,4 alpha , branching enzyme 1
  • Modification:
    Unmodified
  • Storage buffer:
    Aqueous buffered solution containing 0,01M TBS (pH 7,4) with 1% BSA, 0,03% Proclin300 and 50% Glycerol.
  • Molecular weight:
    80 kDa
  • Storage temperature:
    Store at −20 °C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
  • Concentration:
    1 µg/µl
  • Shipping temperature:
    4 °C
  • Immunogen:
    KLH conjugated synthetic peptide derived from human GBE1
  • Tested applications:
    ICC
  • Purification:
    Purified by Protein A
  • Pack type:
    Vial
  • Pk:
    100 µl

Specifications

About this item

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.

Type: Primary
Antigen: GBE1
Clonality: Polyclonal
Clone:
Conjugation: ALEXA FLUOR® 680
Public Immunogen Range: 101-200/702
Host: Rabbit
Isotype: IgG
Reactivity: