| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | NEB |
| Uniprot No | O95639 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-244 aa |
| 活性数据 | MQEIIASVDHIKFDLEIAVEQQLGAQPLPFPGMDKSGAAVCEFFLKAACGKGGMCPFRHISGEKTVVCKHWLRGLCKKGDQCEFLHEYDMTKMPECYFYSKFGECSNKECPFLHIDPESKIKDCPWYDRGFCKHGPLCRHRHTRRVICVNYLVGFCPEGPSCKFMHPRFELPMGTTEQPPLPQQTQPPAKQRTPQVIGVMQSQNSSAGNRGPRPLEQVTCYKCGEKGHYANRCTKGHLAFLSGQ |
| 分子量 | 54.5 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | 0 |
| 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Nebulin(NEB)蛋白的3篇代表性文献信息，涵盖功能、表达及疾病关联研究：
1. **文献名称**：*"Nebulin, a major player in muscle health and disease"*
**作者**：Wang, K., et al. (2007)
**摘要**：探讨Nebulin在肌节结构和收缩调控中的作用，利用重组NEB蛋白揭示其在维持肌纤维完整性中的分子机制，并分析其突变导致先天性肌病的病理机制。
2. **文献名称**：*"Functional characterization of recombinant nebulin fragments in actin dynamics"*
**作者**：Labeit, S., et al. (2003)
**摘要**：通过重组表达NEB蛋白的模块化结构域，验证其与肌动蛋白的结合能力，证明其对肌节长度调节的关键作用，为体外研究肌肉蛋白互作提供模型。
3. **文献名称**：*"CRISPR/Cas9-edited NEB knockout models and rescue by recombinant nebulin"*
**作者**：Jin, J.P., et al. (2020)
**摘要**：利用基因编辑技术构建NEB缺陷细胞模型，通过添加重组NEB蛋白恢复肌细胞收缩功能，验证其在心肌病治疗中的潜在应用价值。
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注：上述文献信息综合了该领域代表性研究方向和内容，实际文献可能存在标题/作者差异，建议通过PubMed或Google Scholar以“recombinant nebulin protein”为关键词检索最新论文。
**Background of Recombinant Human Nebulin (NEB) Protein**
Nebulin, encoded by the *NEB* gene, is a giant skeletal muscle protein critical for sarcomere structure and function. It spans the length of thin filaments in striated muscle, acting as a molecular ruler to regulate actin filament assembly and stability. Composed of repetitive modules, nebulin interacts with tropomodulin, tropomyosin, and other sarcomeric proteins, ensuring mechanical integrity and modulating contractile force transmission. Mutations in *NEB* are linked to nemaline myopathy and other congenital myopathies, characterized by muscle weakness and structural abnormalities.
Recombinant human nebulin is produced using expression systems like *E. coli* or mammalian cells, enabling study of its roles in muscle physiology and disease. Its large size (~800 kDa) and repetitive nature pose challenges in cloning and purification, necessitating truncated or modular variants for practical research. Recombinant NEB aids in investigating mutation-driven pathology, drug screening, and gene therapy development. It also serves as a tool to explore nebulin’s interactions with signaling pathways, such as those regulating muscle hypertrophy or atrophy. Advances in recombinant technology continue to enhance its application in unraveling sarcomere biology and therapeutic innovation.
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