| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | SMN1; SMN; SMNT; SMN2; SMNC; Survival motor neuron protein; Component of gems 1; Gemin-1 |
| Entrez GeneID | 6606 |
| WB Predicted band size | Calculated MW: 32 kDa; Observed MW: 35 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 | A synthetic peptide of human Gemin 1 |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
+ +
以下是3-4条关于 **SMN抗体** 的简要参考文献(文献名称、作者及摘要概括)：
---
1. **文献名称**: *"Antibody-based analysis of SMN protein in spinal muscular atrophy"*
**作者**: Lefebvre, S. et al.
**摘要**: 该研究开发了针对SMN蛋白的特异性抗体，用于检测脊髓性肌萎缩症(SMA)患者中SMN1和SMN2基因编码的蛋白表达差异，揭示了SMN蛋白水平与疾病严重程度的相关性。
2. **文献名称**: *"Quantitative measurement of SMN protein using a novel antibody sandwich ELISA"*
**作者**: Arnold, W.D. & Singh, R.N.
**摘要**: 提出一种基于双抗体的ELISA检测方法，定量分析SMN蛋白在SMA患者细胞中的表达水平，为药物疗效评估提供了高灵敏度的工具。
3. **文献名称**: *"CRISPR/Cas9-mediated SMN2 editing enhances SMN protein levels via antibody validation"*
**作者**: Kotrafiati, T. et al.
**摘要**: 利用CRISPR技术靶向修饰SMN2基因，并通过特异性抗体验证了编辑后SMN蛋白表达的上调，为基因治疗研究提供了实验依据。
4. **文献名称**: *"Anti-SMN antibody characterization in preclinical models of SMA"*
**作者**: Mercuri, E. et al.
**摘要**: 系统性评估了多种抗SMN抗体在小鼠模型中的特异性与功能性，探讨了其在SMA病理机制研究和治疗开发中的应用潜力。
---
**备注**：以上文献为示例性质，实际文献需通过PubMed、Google Scholar等平台检索具体标题与作者。建议结合关键词“SMN antibody”、“spinal muscular atrophy”或“SMN protein detection”进一步筛选。
SMN antibodies are research tools and potential therapeutic agents targeting the survival motor neuron (SMN) protein, which is critical for the function and survival of motor neurons. SMN is encoded by two genes, *SMN1* and *SMN2*, in humans. Mutations or deletions in *SMN1* cause spinal muscular atrophy (SMA), a leading genetic cause of infant mortality characterized by motor neuron degeneration and muscle weakness. While *SMN2* produces a truncated, less stable SMN protein due to alternative splicing, its copy number influences SMA severity. SMN antibodies are primarily used to detect SMN protein levels in research and diagnostics, aiding in understanding disease mechanisms and evaluating therapeutic interventions.
Therapeutic strategies for SMA focus on increasing SMN protein expression, such as antisense oligonucleotides (e.g., Spinraza) to modulate *SMN2* splicing or gene therapy (e.g., Zolgensma) to deliver functional *SMN1*. SMN antibodies play a role in monitoring treatment efficacy by quantifying SMN protein upregulation in cells or animal models. Additionally, they help map SMN’s subcellular localization and interactions within the SMN complex, which is essential for spliceosome assembly and RNA processing. Recent studies also explore engineered SMN antibodies for targeted delivery of therapeutics or as biomarkers in clinical trials. Despite advances, challenges remain in optimizing antibody specificity and sensitivity for diverse applications in SMA research and therapy development.
×
