- ￥880
- Bioss
- bs-7980P
- 2025年10月16日
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- 详细信息
- 技术资料
- 规格：
500ug
| 产品编号 | bs-7980P |
| 英文名称 | GUSB Antibody Blocking Peptide |
| 中文名称 | β葡萄糖醛酸苷酶封闭多肽 |
| 英文别名 | 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. |
| 纯化方法 | HPLC |
| 研究领域 | Metabolism > Pathways and Processes > Metabolic signaling pathways > Alcohol metabolism Signal Transduction > Metabolism > Alcohol metabolism Signal Transduction > Metabolism > Lipid metabolism |
| 亚基 | Homotetramer. |
| 亚细胞定位 | Lysosome. |
| 翻译后修饰 | N-linked glycosylated with 3 to 4 oligosaccharide chains. |
| 相似性 | Belongs to the glycosyl hydrolase 2 family. |
| 功能 | Plays an important role in the degradation of dermatan and keratan sulfates. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
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GUSB Antibody Blocking Peptide(bs-7980P)-500ug
￥880
