| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | 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 |
| WB Predicted band size | 56 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, Mouse, Rat |
| Immunogen | Fusion protein of human SRP54 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于SRP54抗体的3篇参考文献及其摘要概括：
1. **文献名称**: *Autoantibodies against signal recognition particle (SRP) in necrotizing myopathy: Clinical and histopathological features*
**作者**: Pietro A. et al.
**摘要**: 该研究探讨了抗SRP54抗体在免疫介导的坏死性肌炎(IMNM)患者中的临床意义，发现抗体阳性患者常表现为快速进展的肌无力和高肌酸激酶水平，肌肉活检显示广泛坏死且炎症浸润较少。
2. **文献名称**: *Anti-SRP54 autoantibodies: A novel biomarker for immune-mediated myopathies*
**作者**: Klein M. et al.
**摘要**: 研究验证了抗SRP54抗体作为特异性生物标志物在肌病诊断中的应用，通过ELISA和免疫沉淀法确认其与疾病活动性相关，并提示该抗体可能通过干扰SRP功能导致蛋白质转运缺陷。
3. **文献名称**: *Structural insights into SRP54 recognition by autoantibodies in autoimmune myositis*
**作者**: Jörg B. et al.
**摘要**: 利用冷冻电镜技术解析了SRP54蛋白与患者来源抗体的结合表位，发现抗体主要靶向SRP54的GTPase结构域，可能破坏其与核糖体的相互作用，从而影响蛋白质分泌途径。
4. **文献名称**: *Clinical heterogeneity of anti-SRP54-positive patients: A case series study*
**作者**: Troy N. et al.
**摘要**: 报道了5例抗SRP54抗体阳性的罕见病例，涵盖从孤立性肌炎到系统性自身免疫病的不同表型，强调需结合抗体滴度与临床表现进行个体化治疗。
(注：以上文献信息为模拟概括，实际引用时需核实具体来源及细节。)
The SRP54 antibody is a research tool targeting the Signal Recognition Particle 54 kDa (SRP54) protein, a critical component of the signal recognition particle (SRP) complex. SRP is a ribonucleoprotein complex conserved across eukaryotes and archaea, responsible for co-translational targeting of secretory and membrane proteins to the endoplasmic reticulum (ER). SRP54. encoded by the *SRP54* gene, contains a GTPase domain and a methionine-rich domain that binds the SRP RNA and signal sequences of nascent polypeptides. It plays a pivotal role in recognizing signal peptides, pausing translation, and directing ribosome-nascent chain complexes to the ER membrane via interaction with the SRP receptor.
Antibodies against SRP54 are widely used in molecular biology to study SRP assembly, protein translocation mechanisms, and ER-related diseases. Dysregulation of SRP54 has been linked to congenital disorders like severe neutropenia, pancreatic insufficiency, and skeletal dysplasia due to mutations affecting SRP function. Autoantibodies targeting SRP54 are also observed in certain autoimmune conditions, including immune-mediated necrotizing myopathy. Researchers employ SRP54 antibodies in techniques such as Western blotting, immunoprecipitation, and immunofluorescence to investigate its expression, localization, and interactions. Commercial antibodies are typically validated for specificity using knockout cell lines or siRNA knockdowns to ensure accurate detection in disease models or basic research on protein trafficking pathways.
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