β葡萄糖醛酸苷酶抗体

?β葡萄糖醛酸苷酶抗体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 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,β葡萄糖醛酸苷酶抗体 ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. 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, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes.β葡萄糖醛酸苷酶抗体 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:

β葡萄糖醛酸苷酶抗体asd; Beta G1; Beta 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:

●

Rabbit Polyclonal IgG, affinity purified by Protein A.

●

Reacts with: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, .

●

Immunogen: KLH conjugated synthetic peptide derived from human GUSB/beta glucuronidase.

●

β葡萄糖醛酸苷酶抗体Predicted Molecular Weight: 69kDa.

Storage:

Shipped at 4℃, Store 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 dilutions must be determined by the end user.

kl568Rb52S100钙结合蛋白A11(S100A11)多克隆抗体Polyclonal Antibody to S100 Calcium Binding Protein A11 (S100A11)

kl569Hu01P选择素(SELP)多克隆抗体Polyclonal Antibody to Selectin, Platelet (SELP)

kl569Mu01P选择素(SELP)多克隆抗体Polyclonal Antibody to Selectin, Platelet (SELP)

kl569Po01P选择素(SELP)多克隆抗体Polyclonal Antibody to Selectin, Platelet (SELP)

kl569Ra01P选择素(SELP)多克隆抗体Polyclonal Antibody to Selectin, Platelet (SELP)

kl569Rb51P选择素(SELP)多克隆抗体Polyclonal Antibody to Selectin, Platelet (SELP)

kl574Hu01层粘连蛋白α1(LAMα1)多克隆抗体Polyclonal Antibody to Laminin Alpha 1 (LAMa1)

kl574Mu01层粘连蛋白α1(LAMα1)多克隆抗体Polyclonal Antibody to Laminin Alpha 1 (LAMa1)

kl576Hu01磷脂酶A2激活蛋白(PLAA)多克隆抗体Polyclonal Antibody to Phospholikle A2 Activating Protein (PLAA)

kl578Hu01水通道蛋白9(AQP9)多克隆抗体Polyclonal Antibody to Aquaporin 9 (AQP9)

kl579Hu01水通道蛋白1(AQP1)多克隆抗体Polyclonal Antibody to Aquaporin 1, Colton Blood Group (AQP1)

kl580Hu01水通道蛋白2(AQP2)多克隆抗体Polyclonal Antibody to Aquaporin 2, Collecting Duct (AQP2)

kl580Ra01水通道蛋白2(AQP2)多克隆抗体Polyclonal Antibody to Aquaporin 2, Collecting Duct (AQP2)

kl581Mu01水通道蛋白3(AQP3)多克隆抗体Polyclonal Antibody to Aquaporin 3, Gill Blood Group (AQP3)

kl582Hu01水通道蛋白4(AQP4)多克隆抗体Polyclonal Antibody to Aquaporin 4 (AQP4)

kl582Mu01水通道蛋白4(AQP4)多克隆抗体Polyclonal Antibody to Aquaporin 4 (AQP4)

kl582Ra01水通道蛋白4(AQP4)多克隆抗体Polyclonal Antibody to Aquaporin 4 (AQP4)

kl583Hu01水通道蛋白5(AQP5)多克隆抗体Polyclonal Antibody to Aquaporin 5 (AQP5)

kl583Ra01水通道蛋白5(AQP5)多克隆抗体Polyclonal Antibody to Aquaporin 5 (AQP5)

kl584Hu01血红素氧合酶1(HO1)多克隆抗体Polyclonal Antibody to Heme Oxygenase 1, Decycling (HO1)

kl584Mu01血红素氧合酶1(HO1)多克隆抗体Polyclonal Antibody to Heme Oxygenase 1, Decycling (HO1)

kl584Ra01血红素氧合酶1(HO1)多克隆抗体Polyclonal Antibody to Heme Oxygenase 1, Decycling (HO1)