| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | SMA; SMN; SMA1; SMA2; SMA3; SMA4; SMA@; SMNT; BCD541; GEMIN1; TDRD16A; T-BCD541 |
| WB Predicted band size | 32 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | Synthetic peptide of human SMN1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3篇关于SMN1抗体的假设性参考文献示例(注：实际文献需通过学术数据库查询，以下为模拟内容)：
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1. **文献名称**: *Development of a Monoclonal Antibody Specific for SMN1 Protein in Spinal Muscular Atrophy*
**作者**: Smith A, et al.
**摘要**: 本研究开发了一种针对SMN1蛋白的特异性单克隆抗体，通过免疫印迹和免疫组化验证其在区分SMN1与SMN2蛋白异构体中的有效性，为SMA的分子诊断提供了工具。
2. **文献名称**: *SMN1 Antibody-Based Detection of Protein Localization in Motor Neurons*
**作者**: Chen L, et al.
**摘要**: 利用SMN1特异性抗体探究SMN蛋白在运动神经元中的亚细胞定位，发现其与核内小体(Gems)的共定位缺失与SMA严重程度相关，揭示了疾病病理机制。
3. **文献名称**: *Quantitative Analysis of SMN1 Protein Using a Novel ELISA System*
**作者**: Tanaka K, et al.
**摘要**: 开发了一种基于SMN1抗体的高灵敏度ELISA检测方法，可定量患者血液中SMN1蛋白水平，为SMA的早期筛查和治疗监测提供了新策略。
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**提示**：实际研究中，SMN1基因与SMN2高度同源，其编码蛋白序列相同，因此多数抗体针对SMN蛋白通用表位。若需区分SMN1/SMN2.需结合基因型分析。建议通过PubMed或Google Scholar以“SMN1 antibody”或“SMN protein detection”为关键词检索最新文献。
SMN1 antibodies are essential tools in studying spinal muscular atrophy (SMA), a genetic disorder caused by mutations or deletions in the *SMN1* (survival motor neuron 1) gene. The SMN1 protein is critical for the survival and function of motor neurons, facilitating snRNP assembly, RNA splicing, and maintaining neuromuscular junctions. Loss of functional SMN1 leads to SMA, characterized by muscle weakness and degeneration. While humans possess a nearly identical paralog, *SMN2*, its alternative splicing results in low levels of full-length SMN protein, insufficient to compensate for *SMN1* defects.
Antibodies targeting SMN1 are designed to distinguish SMN1-derived protein from SMN2-derived isoforms, leveraging epitopes in regions affected by splicing, such as exon 7. These antibodies enable quantification of SMN protein levels in tissues, cells, or biofluids, aiding SMA diagnosis, monitoring therapeutic responses, and mechanistic studies. Commonly used in techniques like Western blot, immunohistochemistry, and ELISA, they help validate SMN-enhancing therapies, including antisense oligonucleotides (e.g., nusinersen) and gene replacement (e.g., onasemnogene abeparvovec). However, challenges remain in ensuring specificity due to the high homology between SMN1 and SMN2 proteins. Research continues to refine antibody design for improved diagnostic and research applications, supporting advances in SMA treatment and understanding motor neuron biology.
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