嘌呤核苷磷酸化酶抗体

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英文名称  Anti-PNP/Nucleoside phosphorylase

中文名称  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.

浓    度  1mg/1ml

规 格  0.1ml/100μg  0.2ml/200μg

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit

产品类型  一抗    

研究领域  肿瘤 细胞生物 神经生物学 淋巴细胞 t-淋巴细胞  

蛋白分子量  predicted molecular weight: 32kDa

性    状  Lyophilized or Liquid

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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  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

产品应用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500

（石蜡切片需做抗原修复）

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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.

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

产品介绍 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.

嘌呤核苷磷酸化酶抗体Similarity : Belongs to the PNP/MTAP phosphorylase family.

Database links : UniProtKB/Swiss-Prot: P00491.2