Product Description

GBE1 Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS8283-50, BS8283-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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 accumμlation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adμlt polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumμlation 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.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB

Alternate Names: 1, 4-alpha-glucan-branching enzyme, Brancher enzyme, Glycogen-branching enzyme, GBE1

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

Immunogen: Recombinant fμll length Human GBE1.

Dilution: WB 1:500 - 1:2000

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

Specificity: GBE1 polyclonal antibody detects endogenous levels of GBE1 protein.

Molecular Weight: ~ 80 kDa

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

SwissProt: Q04446

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

Research Use Only