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Note: Application as IHC, only suitable for histochemical staining or fluorescence staining of paraffin-embedded sections. Application as ICC/IF, suitable for histochemical or fluorescent staining of frozen sections, as well as chemical and fluorescent staining at the cellular level.
注意：抗体应用为IHC的，抗体只适合于石蜡切片的组化染色或者荧光染色。
抗体应用为IF/ICC的，抗体适合于冰冻切片的组化染色或者荧光染色，以及细胞水平的化学染色和荧光染色。
ABMART实验方案下载
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, 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. 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.
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.
Mouse,Human,Rat,Chicken,Dog,Pig,Cow
WB
WB 1:500-2000
69kD
2990
P08236
Lysosome.
KLH conjugated synthetic peptide derived from human GUSB/beta glucuronidase
Rabbit
Polyclonal
IgG
affinity purified by Protein A
Liquid
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
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程经理：手机18616261485（微信同号）
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徐经理：手机15618191473（微信同号）
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