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GBE1 polyclonal antibody

货号: BS8283

价格：￥ 1140

货期：现货（1-2个工作日）

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说明书 MSDS SHIP

详细信息检测数据实验指南文献

No. :BS8283

Product Name :GBE1 polyclonal antibody

Swiss-Prot :Q04446

Host :Rabbit

Reactivity :Human,Mouse,Rat

Applications :WB

Application_all :WB 1:500 - 1:2000

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.

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

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

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

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

BiowMW :~ 80 kDa

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

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

Immunogen :Recombinant full length Human GBE1.

Conjugate :Unconjugated

Modification :Unmodification

WesternBlot (WB) analysis of GBE1 polyclonal antibody

免疫沉淀（IP）

免疫组织化学（IHC）

免疫荧光（IF）