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Recombinant Human PMP22 protein

  • 中文名: 外周鞘磷脂蛋白22(PMP22)重组蛋白
  • 别    名: PMP22;GAS3;Peripheral myelin protein 22
货号: PA1000-9162
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点PMP22
Uniprot NoQ01453
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间1-160aa
氨基酸序列MLLLLLSIIVLHVAVLVLLFVSTIVSQWIVGNGHATDLWQNCSTSSSGNV HHCFSSSPNEWLQSVQATMILSIIFSILSLFLFFCQLFTLTKGGRFYITG IFQILAGLCVMSAAAIYTVRHPEWHLNSDYSYGFAYILAWVAFPLALLSG VIYVILRKRE
预测分子量45 kDa
蛋白标签His tag N-Terminus
缓冲液PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
稳定性 & 储存条件Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt.
Reconstituted protein solution can be stored at 2-8°C for 2-7 days.
Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months.
复溶Always centrifuge tubes before opening.Do not mix by vortex or pipetting.
It is not recommended to reconstitute to a concentration less than 100μg/ml.
Dissolve the lyophilized protein in distilled water.
Please aliquot the reconstituted solution to minimize freeze-thaw cycles.

参考文献

1. **文献名称**: "Expression and purification of recombinant human peripheral myelin protein 22 (PMP22)"

**作者**: D'Urso D, et al.

**摘要**: 该研究描述了在细菌系统中高效表达和纯化重组人PMP22蛋白的方法,并验证了其与天然蛋白的结构相似性,为后续功能研究提供基础。

2. **文献名称**: "Structural characterization of PMP22 recombinant protein in lipid bilayers by solid-state NMR"

**作者**: Wang Y, et al.

**摘要**: 利用固态核磁共振技术解析重组PMP22蛋白在脂双层中的构象,揭示了其跨膜结构域动态特性,探讨了突变导致周围神经病变的分子机制。

3. **文献名称**: "PMP22 aggregation and toxicity in Charcot-Marie-Tooth disease models using recombinant protein expression"

**作者**: Fortun J, et al.

**摘要**: 通过细胞模型研究重组PMP22蛋白的异常聚集行为,发现其错误折叠与内质网应激相关,为CMT1A型疾病的病理机制提供实验依据。

4. **文献名称**: "Development of PMP22-specific nanobodies using recombinant antigen immunization"

**作者**: Seyer A, et al.

**摘要**: 采用重组PMP22蛋白免疫骆驼科动物,筛选出高特异性纳米抗体,为未来开发针对PMP22相关神经病变的诊断和治疗工具奠定基础。

注:以上为虚拟文献,实际文献需通过PubMed/Google Scholar检索关键词(如"PMP22 recombinant protein"、"expression"、"structure"、"aggregation")获取。

背景信息

**Background of PMP22 Recombinant Protein**

The Peripheral Myelin Protein 22 (PMP22) is a tetraspan membrane glycoprotein crucial for the formation and maintenance of myelin sheaths in the peripheral nervous system. Expressed predominantly by Schwann cells, PMP22 accounts for 2–5% of total myelin protein and plays a vital role in cell membrane stability, intracellular trafficking, and Schwann cell differentiation. Its gene, located on human chromosome 17p12. is tightly regulated during development and myelination.

Mutations or dysregulation of PMP22 are linked to several hereditary neuropathies. A 1.4-Mb duplication in the PMP22 gene causes Charcot-Marie-Tooth disease type 1A (CMT1A), the most common inherited demyelinating neuropathy, while deletions or point mutations result in Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) or severe early-onset neuropathies. These conditions highlight PMP22's dosage sensitivity and its critical role in nerve function.

Recombinant PMP22 protein, produced via heterologous expression systems (e.g., *E. coli*, mammalian cells), enables detailed study of its structure, interactions, and pathogenic mechanisms. Purified recombinant PMP22 retains functional epitopes, making it valuable for antibody development, protein-protein interaction assays, and drug screening. Researchers use it to model PMP22 aggregation in neuropathies and to explore therapeutic strategies, such as small molecule chaperones or gene therapy.

Despite challenges in expressing this hydrophobic protein, advances in solubilization and refolding techniques have improved recombinant PMP22 yield and stability. Ongoing studies focus on elucidating its role in intracellular signaling and myelin maintenance, with the goal of translating findings into targeted therapies for PMP22-related disorders.

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