产品详情
简单介绍：
Anti-GBE1抗体产品质量稳定，实验效果明显，货期快，价格优惠，欢迎垂询订购！我公司长期供应**组化抗体、WB抗体、**组化试剂盒和抗体试验所需全部相关试剂、荧光标记抗体、单克隆抗体、多克隆抗体、各种标记的二抗IgG/IgM/IgD/IgA等科研实验抗体。Anti-GBE1抗体用于**组化实验,WB实验,相应的标记抗体有HRP标记抗体,FITC标记,BIO等。
详情介绍：
Rabbit Anti-GBE1
Cat. Number:
Anti-GBE1抗体KL-13300R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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
Anti-GBE1抗体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 ***** 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.
Also known as:
1,4 alpha glucan branching
Anti-GBE1抗体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.
Specificity:
●
Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Rabbit, .
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Immunogen: KLH conjugated synthetic peptide derived from human GBE1.
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Predicted Molecular Weight: 80kDa.
Storage:
Shipped at 4℃, Store at -20℃
Anti-GBE1抗体(Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions Anti-GBE1抗体must be determined by the end user.
Not yet tested in other applications.
Optimal working dilutions Anti-GBE1抗体must be determined by the end user.
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