GBE1 polyclonal antibody

• Background :

  GBE1 (glucan (1,4-alpha-), branching enzyme 1) 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.

• Structure Composition :

  Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

• Product Name Alternative :

  1,4-alpha-glucan-branching enzyme; Brancher enzyme; Glycogen-branching enzyme; GBE1

• Swiss Prot :

  Q04446

• Reactivity :

  Human, Mouse, Rat

• Immunogen :

  Recombinant full length Human GBE1.

• Conjugation :

  Unconjugated

• Applications :

  WB

• Dilution :

  WB 1:500 - 1:2000

• Purity :

  The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

• Modification :

  Unmodification

• Molecular Weight :

  ~ 80 kDa

• Storage Conditions :

  Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

• Notes :

  For research use only, not for use in diagnostic procedure.

• Specificity :

  GBE1 polyclonal antibody detects endogenous levels of GBE1 protein.

• Applications Notes :

  WesternBlot (WB) analysis of GBE1 polyclonal antibody

• Host or Source :

  Rabbit

• CAS Number :

  9007-83-4