產(chǎn)品詳情
簡(jiǎn)單介紹：
Anti-beta glucuronidase抗體產(chǎn)品質(zhì)量穩(wěn)定，實(shí)驗(yàn)效果明顯，貨期快，價(jià)格優(yōu)惠，歡迎垂詢訂購！我公司長期供應(yīng)**組化抗體、WB抗體、**組化試劑盒和抗體試驗(yàn)所需全部相關(guān)試劑、熒光標(biāo)記抗體、單克隆抗體、多克隆抗體、各種標(biāo)記的二抗IgG/IgM/IgD/IgA等科研實(shí)驗(yàn)抗體。Anti-beta glucuronidase抗體用于**組化實(shí)驗(yàn),WB實(shí)驗(yàn),相應(yīng)的標(biāo)記抗體有HRP標(biāo)記抗體,FITC標(biāo)記,BIO等。
詳情介紹：
Rabbit Anti-beta glucuronidase
Cat. Number:
Anti-beta glucuronidase抗體KL-7980R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Defects in GUSB are theAnti-beta glucuronidase抗體 cause of mucopolysaccharidosis type 7 (MPS7) ; also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into *****hood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment. Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis,Anti-beta glucuronidase抗體 a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
Also known as:
asd; Beta G1; Beta Anti-beta glucuronidase抗體glucuronidase; Beta-G1; Beta-glucuronidase; BG; BGLR; BGLR_HUMAN; Glucuronidase beta; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; GUSB; Gut; MPS7; Ac2-223.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, .
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Immunogen: KLH conjugated synthetic peptide derived from human GUSB/beta glucuronidase.
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Predicted Molecular Weight: 69kDa.
Storage:
Shipped at 4℃, StoreAnti-beta glucuronidase抗體 at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal workingAnti-beta glucuronidase抗體 dilutions must be determined by the end user.
Not yet tested in other applications.
Optimal workingAnti-beta glucuronidase抗體 dilutions must be determined by the end user.
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