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货号： bs-11739R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11739R-Bio
- 英文名称
- Anti-Nucleoside phosphorylase/Biotin
- 中文名称
- 生物素标记的嘌呤核苷磷酸化酶抗体
- 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.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 神经生物学 淋巴细胞 t-淋巴细胞
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
- 产品应用
- ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 32kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PNP/Nucleoside phosphorylase
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
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.
Function:
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.
Subunit:
Homotrimer.
Subcellular Location:
Cytoplasm, cytoskeleton (By similarity).Cytoplasm
Tissue Specificity:
Expressed in red blood cells; overexpressed inred blood cells (cytoplasm) of patients with hereditarynon-spherocytic hemolytic anemia of unknown etiology.
DISEASE:
Defects in PNP are the cause of purine nucleosidephosphorylase deficiency (PNPD) [MIM:613179]. It leads to a severeT-cell immunodeficiency with neurologic disorder in children.
Similarity:
Belongs to the PNP/MTAP phosphorylase family.
Database links:Entrez Gene: 4860Human
Omim: 164050Human
SwissProt: P00491Human
Unigene: 75514Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.