| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PRPH |
| Uniprot No | P41219 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-470aa |
| 氨基酸序列 | MSHHPSGLRA GFSSTSYRRT FGPPPSLSPG AFSYSSSSRF SSSRLLGSAS PSSSVRLGSF RSPRAGAGAL LRLPSERLDF SMAEALNQEF LATRSNEKQE LQELNDRFAN FIEKVRFLEQ QNAALRGELS QARGQEPARA DQLCQQELRE LRRELELLGR ERDRVQVERD GLAEDLAALK QRLEEETRKR EDAEHNLVLF RKDVDDATLS RLELERKIES LMDEIEFLKK LHEEELRDLQ VSVESQQVQQ VEVEATVKPE LTAALRDIRA QYESIAAKNL QEAEEWYKSK YADLSDAANR NHEALRQAKQ EMNESRRQIQ SLTCEVDGLR GTNEALLRQL RELEEQFALE AGGYQAGAAR LEEELRQLKE EMARHLREYQ ELLNVKMALD IEIATYRKLL EGEESRISVP VHSFASLNIK TTVPEVEPPQ DSHSRKTVLI KTIETRNGEV VTESQKEQRS ELDKSSAHSY |
| 预测分子量 | 53,6 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. |
以下是关于PRPH(Peripherin)重组蛋白的3篇参考文献，按研究主题分类整理：
### 1. **重组PRPH蛋白的制备与聚集机制研究**
**文献名称**：*Expression and purification of recombinant human peripherin for aggregation studies*
**作者**：Robertson J, et al.
**摘要**：研究通过大肠杆菌系统表达重组人源PRPH蛋白，优化纯化流程，并发现其在体外易形成类似神经退行性疾病中的异常聚集体，为探究PRPH在ALS中的作用机制提供模型。
### 2. **PRPH与神经轴突发育的功能探索**
**文献名称**：*Role of peripherin in axonal outgrowth revealed by recombinant protein interaction assays*
**作者**：Larivière RC, et al.
**摘要**：利用重组PRPH蛋白进行体外结合实验，证实其通过与神经生长锥蛋白相互作用调控轴突延伸，提示PRPH在神经发育中的关键角色。
### 3. **疾病相关突变体的重组表达分析**
**文献名称**：*Biophysical characterization of ALS-linked peripherin mutants using recombinant proteins*
**作者**：Corrado L, et al.
**摘要**：通过表达携带ALS相关突变的PRPH重组蛋白，发现特定突变(如p.P345S)导致蛋白稳定性下降和异常二聚化，从结构角度解释突变如何引发神经病理。
---
#### 关键研究趋势：
- **技术方法**：多采用原核表达系统(如大肠杆菌)制备重组PRPH，通过His标签简化纯化。
- **疾病关联**：聚焦PRPH异常聚集与ALS、CMT等神经疾病的分子关联。
- **突变分析**：利用重组蛋白技术解析致病突变的生化效应。
如需扩展至4篇或深入某类研究可进一步补充。
Peripherin (PRPH), a type III intermediate filament protein, plays a structural and regulatory role in neuronal cells. Encoded by the *PRPH* gene in humans, it is predominantly expressed in the peripheral nervous system (PNS) and subsets of central nervous system (CNS) neurons. Peripherin contributes to cytoskeletal integrity, cellular signaling, and axon outgrowth, and is involved in maintaining mechanical stability during neurodevelopment and regeneration. Its misfolding or aggregation has been linked to neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and Charcot-Marie-Tooth disease.
Recombinant PRPH protein is produced via genetic engineering, typically using bacterial (e.g., *E. coli*) or mammalian expression systems. This allows large-scale, purified protein generation for studying its biological functions and pathological mechanisms. Researchers employ recombinant PRPH to investigate its interactions with other cytoskeletal components (e.g., neurofilaments) and its role in stress responses. It also serves as an antigen for antibody development in diagnostic assays and as a tool to model protein aggregation in neurodegeneration.
Current studies focus on its dual role: while peripherin supports neuronal resilience under normal conditions, abnormal forms may trigger neurotoxicity. Mutations or dysregulation of *PRPH* are associated with disrupted axonal transport and impaired nerve regeneration. Recombinant protein-based models help identify therapeutic targets to mitigate peripherin-related pathologies. Additionally, engineered PRPH variants are explored in biomaterial research for nerve tissue engineering. Despite progress, challenges remain in understanding its isoform-specific functions and post-translational modifications, underscoring the need for further structural and functional characterization.
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