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- 技术资料
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500ug
| 产品编号 | bs-11739P |
| 英文名称 | PNP Antibody Blocking Peptide |
| 中文名称 | 嘌呤核苷磷酸化酶封闭多肽 |
| 英文别名 | Inosine phosphorylase; MGC117396; MGC125915; MGC125916; NP; Np1; Nucleoside phosphorylase; PNP; Pnp1; PNPH_HUMAN; PRO1837; PUNP; Purine nucleoside orthophosphate ribosyltransferase; Purine nucleoside phosphorylase; FLJ94043; FLJ97288. |
| 纯化方法 | HPLC |
| 研究领域 | Neuroscience > Neurology process > Neurodegenerative disease |
| 亚基 | Homotrimer. |
| 亚细胞定位 | Cytoplasm, cytoskeleton (By similarity).Cytoplasm |
| 组织特异性 | Expressed in red blood cells; overexpressed inred blood cells (cytoplasm) of patients with hereditarynon-spherocytic hemolytic anemia of unknown etiology. |
| 相似性 | Belongs to the PNP/MTAP phosphorylase family. |
| 功能 | Defects in PNP are the cause of purine nucleoside phosphorylase deficiency (PNP deficiency) [MIM:613179]. It leads to a severe T-cell immunodeficiency with neurologic disorder in children. |
| 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 | Purine nucleoside phosphorylase (PNP), also designated inosine phosphorylase, forms a homotrimer. It belongs to the PNP/MTAP phosphorylase family of proteins. Human PNP catalyzes the reversible phosphorolysis of ribonucleosides and 2’-deoxyribonucleosides with specificity for guanine, hypoxanthine, and their analogs. PNP deficiency is a rare autosomal recessive genetic disease associated with a severe defect in T-lymphocyte function and neurologic disorder in children, comprising four percent of combined immunodeficiency cases. Children with PNP deficiency are highly prone to infections, autoimmune disorders, neurological impairment, and cancer. |
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PNP Antibody Blocking Peptide(bs-11739P)-500ug
¥880







