GUSB/beta glucuronidaseβ葡萄糖醛酸苷酶抗体

本公司经销GUSB/beta glucuronidase，β葡萄糖醛酸苷酶抗体，克隆类型为polyclonal，宿主来源是Rabbit，GUSB/beta glucuronidaseβ葡萄糖醛酸苷酶抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-7980R
英文名称：Anti-GUSB/beta glucuronidase
中文名称：β葡萄糖醛酸苷酶抗体
其他名称：名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.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 69kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, pig, cow, dog, chk
产品介绍：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.Subunit : Homotetramer.Subcellular Location : Lysosome.Similarity : Belongs to the glycosyl hydrolase 2 family.