产品详情
简单介绍：
Anti-beta glucuronidase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-beta glucuronidase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-beta glucuronidase
Cat. Number:
Anti-beta glucuronidase抗体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 the
Anti-beta glucuronidase抗体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抗体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抗体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℃, Store
Anti-beta glucuronidase抗体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 working Anti-beta glucuronidase抗体dilutions must be determined by the end user.
Not yet tested in other applications.
Optimal working Anti-beta glucuronidase抗体dilutions must be determined by the end user.
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kl224Mu01 胃泌素(GT)多克隆抗体 Polyclonal Antibody to Gastrin (GT)
kl224Ra01 胃泌素(GT)多克隆抗体 Polyclonal Antibody to Gastrin (GT)
kl224Ca01 胃泌素(GT)多克隆抗体 Polyclonal Antibody to Gastrin (GT)
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kl044Ca01 生长**(GH)多克隆抗体 Polyclonal Antibody to Growth Hormone (GH)
kl044Po01 生长**(GH)多克隆抗体 Polyclonal Antibody to Growth Hormone (GH)
kl044Eq01 生长**(GH)多克隆抗体 Polyclonal Antibody to Growth Hormone (GH)
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kl266Mu01 胰高血糖素(GC)多克隆抗体 Polyclonal Antibody to Glucagon (GC)
kl266Ra01 胰高血糖素(GC)多克隆抗体 Polyclonal Antibody to Glucagon (GC)
kl438Hu01 促生长**释放**(GHRH)多克隆抗体 Polyclonal Antibody to Growth Hormone Releasing Hormone (GHRH)
kl438Mu01 促生长**释放**(GHRH)多克隆抗体 Polyclonal Antibody to Growth Hormone Releasing Hormone (GHRH)
kl438Ra01 促生长**释放**(GHRH)多克隆抗体 Polyclonal Antibody to Growth Hormone Releasing Hormone (GHRH)
kl438Po01 促生长**释放**(GHRH)多克隆抗体 Polyclonal Antibody to Growth Hormone Releasing Hormone (GHRH)
kl438Bo01 促生长**释放**(GHRH)多克隆抗体 Polyclonal Antibody to Growth Hormone Releasing Hormone (GHRH)
kl438Cp01 促生长**释放**(GHRH)多克隆抗体 Polyclonal Antibody to Growth Hormone Releasing Hormone (GHRH)
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