供货周期: | 现货 |
品牌: | 康朗生物 |
规格: | 0.1ml/0.2ml |
货号: | KL7980R |
CAS号: |
?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.
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