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  4. Recombinant Mouse Peripheral myelin protein 22 (Pmp22)

Recombinant Mouse Peripheral myelin protein 22 (Pmp22)

  • 中文名称：
    小鼠Pmp22重组蛋白
  • 货号：
    CSB-CF018241MO
  • 规格：
  • 来源：
    in vitro E.coli expression system
  • 其他：

产品详情

  • 基因名：
    Pmp22
  • Uniprot No.：
  • 别名：
    Pmp22; Gas-3; Gas3; Pmp-22; Peripheral myelin protein 22; PMP-22; Growth arrest-specific protein 3; GAS-3
  • 种属：
    Mus musculus (Mouse)
  • 蛋白长度：
    Full length protein
  • 表达区域：
    1-161
  • 氨基酸序列
    MLLLLLGILFLHIAVLVLLFVSTIVSQWLVGNGHTTDLWQNCTTSALGAVQHCYSSSVSE WLQSVQATMILSVIFSVLALFLFFCQLFTLTKGGRFYITGFFQILAGLCVMSAAAIYTVR HSEWHVNTDYSYGFAYILAWVAFPLALLSGIIYVILRKREL
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 10xHis-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    Might be involved in growth regulation, and in myelinization in the peripheral nervous system.
  • 基因功能参考文献：
    1. selective suppression of the Pmp22 mutant allele by non-viral delivery of siRNA alleviates the demyelinating neuropathic phenotypes of Charcot-Marie-Tooth disease in vivo PMID: 28108290
    2. We discovered that Tead1 and co-activators Yap and Taz are required for Pmp22 expression, as well as for the expression of Egr2 Tead1 directly binds Pmp22 and Egr2 enhancers early in development and Tead1 binding is induced during myelination, correlating with Pmp22 expression. The data identify Tead1 as a novel regulator of Pmp22 expression during development in concert with Sox10 and Egr2 PMID: 27288457
    3. The basal lamina and PMP22 act in concert to contribute to a resilience and integrity of peripheral nerves at the single fibre level. PMID: 25446378
    4. A role was identified for PMP22 in the linkage of the actin cytoskeleton with the plasma membrane. PMID: 25429154
    5. This study demonistrated that Paranodal dysmyelination in peripheral nerves of Trembler mice. PMID: 24446165
    6. This study showed that a number of ongoing pathogenic mechanisms contribute to the progression of the neuropathy in C22 mice, which initiates with abnormal expression of PMP22. PMID: 24175617
    7. This study revealed a novel mechanism by which PMP22 deficiency affects nerve conduction not through removal of myelin, but through disruption of myelin junctions PMID: 24339129
    8. This study showed that mouse PMP22 is palmitoylated at C85 and mutating C85S abolishes PMP22 palmitoylation. PMID: 23127255
    9. Peripheral myelin protein 22 (PMP22) performs distinct actions on the formation, maturation, degeneration and regeneration of sciatic nerve myelin sheath. PMID: 21824506
    10. Egr2 and Sox10 activity are directly involved in mediating the developmental induction of Pmp22 expression through an intronic enhancer. PMID: 21411665
    11. The results of this study demonstrated that a function of Pmp22 is to protect the nerve from mechanical injury. PMID: 20071523
    12. Part of the PMP22 gene contains the necessary information to mirror the endogenous expression pattern in peripheral nerves during development and regeneration and in mouse models of demyelination due to genetic lesions. PMID: 12056842
    13. Association of calnexin ex vivo: a basis for "gain-of-function" ER diseases. PMID: 12119418
    14. Aggresome formation has now been observed with two mutant PMP22s, the Tr- and TrJ-PMP22 when the proteasome is inhibited. two pathways of PMP22 degradation are present. PMID: 12127149
    15. Mutant pmp22 from less severely affected mutants occurs in large aggregates, while that from more severely affected mutants occurs in a diffuse perinuclear pattern. Pmp22 aggregates may be protective in this form of peripheral neuropathy. PMID: 12359155
    16. Multiple distinct signaling pathways regulating Pmp22 expression in myelination as well as in neurons converge on distinct segments of the PMP22 promoter region. PMID: 14664827
    17. Recessive mutations were uniquely distinguished from dominant mutations by both the low potential for aggregation and their trafficking to the cell surface. PMID: 15474367
    18. Increased expression of genes involved in cell cycle regulation and DNA replication is characteristic and specific for early development in Pmp22-deficient mice, supporting a primary function of PMP22 in the regulation of Schwann cell proliferation. PMID: 15755691
    19. PMP22 is a binding partner in the integrin alpha6beta4/laminin complex and is involved in mediating the interaction of Schwann cells with the extracellular environment. PMID: 16436605
    20. The beneficial effects of autophagy and chaperones in preventing the accumulation of misfolded PMP22 are additive and provide a potential avenue for therapeutic approaches in hereditary neuropathies linked to PMP22 mutations. PMID: 17174099
    21. Data show that Med25 is coordinately expressed with Pmp22 gene dosage and expression in transgenic mice and rats, and suggest a potential role of this protein in the molecular etiology of Charcot-Marie-Tooth disease. PMID: 19290556

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  • 相关疾病：
    A defect in Pmp-22 is the cause of trembler (tr) phenotype. Trembler mice show a Schwann cells defect characterized by severe hypomyelination and continuing Schwann cells proliferation throughout life.
  • 亚细胞定位：
    Cell membrane; Multi-pass membrane protein.
  • 蛋白家族：
    PMP-22/EMP/MP20 family
  • 组织特异性：
    Schwann cells of the peripheral nervous system.
  • 数据库链接：

    KEGG: mmu:18858

    STRING: 10090.ENSMUSP00000018361

    UniGene: Mm.1237