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Rabbit Anti-Nucleoside phosphorylase  antibody (bs-11739R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-11739R
英文名稱 Rabbit Anti-Nucleoside phosphorylase  antibody
中文名稱 嘌呤核苷磷酸化酶抗體
別    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.  
研究領域 腫瘤  細胞生物  神經生物學  淋巴細胞  t-淋巴細胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 32kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PNP/Nucleoside phosphorylase: 201-280/289 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
P00491

Gene ID:
4860

Database links:

Entrez Gene: 4860 Human

Omim: 164050 Human

SwissProt: P00491 Human

Unigene: 75514 Human



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