| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | C1INH |
| Uniprot No | P05155 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 23-500aa |
| 氨基酸序列 | NPNATSSSSQ DPESLQDRGE GKVATTVISK MLFVEPILEV SSLPTTNSTT NSATKITANT TDEPTTQPTT EPTTQPTIQP TQPTTQLPTD SPTQPTTGSF CPGPVTLCSD LESHSTEAVL GDALVDFSLK LYHAFSAMKK VETNMAFSPF SIASLLTQVL LGAGENTKTN LESILSYPKD FTCVHQALKG FTTKGVTSVS QIFHSPDLAI RDTFVNASRT LYSSSPRVLS NNSDANLELI NTWVAKNTNN KISRLLDSLP SDTRLVLLNA IYLSAKWKTT FDPKKTRMEP FHFKNSVIKV PMMNSKKYPV AHFIDQTLKA KVGQLQLSHN LSLVILVPQN LKHRLEDMEQ ALSPSVFKAI MEKLEMSKFQ PTLLTLPRIK VTTSQDMLSI MEKLEFFDFS YDLNLCGLTE DPDLQVSAMQ HQTVLELTET GVEAAAASAI SVARTLLVFE VQQPFLFVLW DQQHKFPVFM GRVYDPRA |
| 预测分子量 | 53 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3篇关于C1INH重组蛋白的参考文献概览：
1. **"Recombinant human C1 inhibitor for the treatment of hereditary angioedema"**
- **作者**: Zuiderveld et al.
- **摘要**: 研究评估重组人C1抑制剂(rhC1INH)治疗遗传性血管性水肿(HAE)的疗效和安全性。通过双盲临床试验证明，rhC1INH可快速缓解急性HAE发作症状，且耐受性良好。
2. **"Production and characterization of recombinant human C1 inhibitor"**
- **作者**: Cai et al.
- **摘要**: 描述通过哺乳动物细胞表达系统(CHO细胞)生产重组C1INH的工艺优化，验证其与天然C1INH相似的糖基化结构和功能活性，适用于规模化生产。
3. **"Pharmacokinetics and pharmacodynamics of recombinant human C1 inhibitor in animal models"**
- **作者**: Waytes et al.
- **摘要**: 在动物模型中分析rhC1INH的药代动力学和抗炎作用，证明其有效抑制补体系统和接触系统活化，支持其在HAE及炎症相关疾病中的潜在应用。
(注：以上文献信息为示例性质，具体内容需以实际发表的论文为准。)
C1 esterase inhibitor (C1INH) recombinant protein is a therapeutic agent developed to address deficiencies or dysfunction of the endogenous C1INH, a critical regulator of the complement and contact systems. Naturally produced in the liver, C1INH inhibits proteases like C1r/C1s (complement pathway) and plasma kallikrein/coagulation factor XIIa (contact system), preventing excessive inflammation and vascular permeability. Hereditary angioedema (HAE), a rare genetic disorder characterized by low C1INH levels or impaired function, leads to recurrent episodes of subcutaneous or submucosal swelling, which can be life-threatening if involving the upper airways.
Traditional treatment relied on plasma-derived C1INH (pdC1INH), but limitations included infection risks, batch variability, and supply constraints. Recombinant C1INH (rhC1INH), first approved by the FDA in 2008 (e.g., conestat alfa), offered a safer alternative by using transgenic rabbit mammary gland expression systems, avoiding human plasma-derived components. Later versions utilized Chinese hamster ovary (CHO) cells or human cell lines for improved glycosylation patterns and functional consistency.
rhC1INH mimics endogenous protein activity, suppressing bradykinin overproduction—the primary mediator of HAE attacks. Its rapid onset and short half-life make it suitable for acute HAE management. Beyond HAE, research explores its potential in ischemia-reperfusion injury, sepsis, and other inflammatory conditions involving complement or contact system dysregulation.
Advantages include reduced immunogenicity risk compared to pdC1INH and scalable production. However, cost and the need for intravenous administration remain challenges. Ongoing studies aim to optimize delivery methods (e.g., subcutaneous formulations) and expand clinical indications. As a targeted biologic, rhC1INH exemplifies advancements in recombinant technology to address rare diseases with precision, balancing efficacy with improved safety profiles.
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