PNP Antibody Blocking Peptide(bs-11739P)-500ug

PNP Antibody Blocking Peptide(

bs-11739P)-500ug
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  • bs-11739P
  • 2025年10月16日
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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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