產品詳情
簡單介紹:
Anti-GBE1抗體產品質量穩定,實驗效果明顯,貨期快,價格優惠,歡迎垂詢訂購!我公司長期供應**組化抗體、WB抗體、**組化試劑盒和抗體試驗所需全部相關試劑、熒光標記抗體、單克隆抗體、多克隆抗體、各種標記的二抗IgG/IgM/IgD/IgA等科研實驗抗體。Anti-GBE1抗體用于**組化實驗,WB實驗,相應的標記抗體有HRP標記抗體,FITC標記,BIO等。
詳情介紹:
Rabbit Anti-GBE1
Cat. Number:
Anti-GBE1抗體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抗體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抗體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.
●
Predicted Molecular Weight: 80kDa.
Storage:
Shipped at 4℃, Store at -20℃Anti-GBE1抗體 (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抗體must be determined by the end user.
Not yet tested in other applications.
Optimal working dilutions Anti-GBE1抗體must be determined by the end user.
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