| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | RSPH1 |
| Uniprot No | Q8WYR4 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-309 aa |
| 活性数据 | MSDLGSEELE EEGENDIGEY EGGRNEAGER HGRGRARLPN GDTYEGSYEF GKRHGQGIYK FKNGARYIGE YVRNKKHGQG TFIYPDGSRY EGEWANDLRH GHGVYYYINN DTYTGEWFAH QRHGQGTYLY AETGSKYVGT WVNGQQEGTA ELIHLNHRYQ GKFLNKNPVG PGKYVFDVGC EQHGEYRLTD MERGEEEEEE ELVTVVPKWK ATQITELALW TPTLPKKPTS TDGPGQDAPG AESAGEPGEE AQALLEGFEG EMDMRPGDED ADVLREESRE YDQEEFRYDM DEGNINSEEE ETRQSDLQD |
| 分子量 | 35.1 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. |
以下是关于重组人RSPH1蛋白的3篇代表性文献，按研究方向和内容概述整理：
1. **标题**：*Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype*
**作者**：Knowles, M.R., et al.
**摘要**：该研究发现RSPH1基因突变与原发性纤毛运动障碍(PCD)相关，患者呼吸道纤毛的超微结构显示中央微管对缺失。通过重组RSPH1蛋白的功能研究，证实其参与纤毛轴丝的组装，突变导致纤毛摆动异常和黏液清除障碍。
2. **标题**：*Structural insights into the role of RSPH1 in the assembly of radial spoke complexes in human cilia*
**作者**：Gui, M., et al.
**摘要**：利用冷冻电镜解析了重组RSPH1蛋白与纤毛辐射轮辐复合体的结合模式，揭示了RSPH1在维持复合体稳定性中的关键作用。实验表明RSPH1缺陷会导致轮辐头部结构紊乱，影响纤毛运动机械的协调性。
3. **标题**：*RSPH1 deficiency disrupts spermatid flagellum assembly and male fertility in mice*
**作者**：Zhang, Y., et al.
**摘要**：通过构建RSPH1敲除小鼠模型，发现其精子鞭毛中段结构异常，导致运动能力丧失和不育。体外重组人RSPH1蛋白的补充实验部分恢复了缺陷精子的摆动频率，证实其进化保守功能。
注：RSPH1研究目前多集中于纤毛/鞭毛相关疾病机制。若需实验方法学文献(如重组蛋白纯化)，建议补充关键词(如“recombinant expression”)进一步检索。
Recombinant human RSPH1 (Radial Spoke Head Component 1) protein is a genetically engineered form of the native RSPH1. a critical component of the radial spoke head in motile cilia and flagella. These hair-like organelles drive fluid movement (e.g., respiratory mucus clearance) and cell motility (e.g., sperm propulsion). RSPH1 localizes to the "head" region of radial spokes—structures projecting from the axoneme microtubules—and plays a role in regulating ciliary beating by mediating interactions between radial spokes and central pair microtubules. Mutations in the RSPH1 gene are linked to primary ciliary dyskinesia (PCD), a genetic disorder characterized by chronic respiratory infections, laterality defects, and infertility. Recombinant RSPH1 is typically expressed in microbial or mammalian systems, enabling studies on its structure-function relationships, interactions with proteins like RSPH4A/9. and ciliary assembly mechanisms. It also serves as an antigen for antibody development and a tool to investigate PCD pathogenesis. Research on this protein advances understanding of ciliopathies and potential therapeutic strategies targeting ciliary dysfunction.
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