GBE1 Polyclonal Antibody

• 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.

• 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.

• Gene ID :

  2632

• Cellular Locus :

  Cytoplasm

• Host :

  Rabbit

• Immunogen :

  101-200/702

• Target :

  GBE1

• Clonality :

  Polyclonal

• Isotype :

  IgG

• Conjugation :

  Unconjugated

• Source :

  KLH conjugated synthetic peptide derived from human GBE1

• Applications :

  WB, ELISA, IHC-P, IHC-F, IF (IHC-P), IF (IHC-F), IF (ICC)

• Purification :

  Purified by Protein A.

• Concentration :

  1µg/µl

• Dilution :

  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)

• Buffer :

  0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

• Modification :

  Unmodified

• Storage Conditions :

  Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.

• Gene ID URL :

  2632

• Predicted Cross Reactivity :

  Human, Mouse, Rat, Rabbit