| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 1/20-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Calmitin; Calmitine; Calsequestrin; Calsequestrin-1; CASQ; Casq1; PDIB1;;Calsequestrin 1 |
| WB Predicted band size | Calculated MW: 45 kDa ; Observed MW: 63 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 | A synthesized peptide derived from human Calsequestrin 1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
+ +
以下是关于Calsequestrin1(CASQ1)抗体的3篇参考文献的简要总结：
---
1. **文献名称**: *"Calsequestrin 1 is a key determinant of skeletal muscle function and disease susceptibility"*
**作者**: Paolini, C. et al. (2005)
**摘要**: 该研究通过基因敲除小鼠模型，利用CASQ1特异性抗体进行免疫印迹和免疫组化分析，揭示了CASQ1在骨骼肌钙离子稳态中的核心作用。实验表明，CASQ1缺失会导致肌浆网钙储存能力下降，从而引发肌肉收缩功能障碍。
---
2. **文献名称**: *"Characterization of calsequestrin isoforms in skeletal and cardiac muscle using monoclonal antibodies"*
**作者**: Volpe, P. et al. (1994)
**摘要**: 研究团队开发了针对CASQ1和CASQ2(心肌亚型)的单克隆抗体，并通过免疫印迹和免疫荧光验证其特异性。实验证明，CASQ1抗体仅识别骨骼肌组织中的蛋白，为后续区分两种亚型的功能研究提供了工具基础。
---
3. **文献名称**: *"Calsequestrin 1 mutations in human skeletal muscle disease: implications for calcium regulation"*
**作者**: Treves, S. et al. (1997)
**摘要**: 该研究在恶性高热(MH)患者中检测到CASQ1基因突变，并利用CASQ1抗体进行患者肌肉活检组织的蛋白表达分析。结果显示，突变导致CASQ1蛋白稳定性降低，可能通过影响钙释放参与疾病发生。
---
**备注**：以上文献主要涉及CASQ1抗体的应用，包括功能研究、亚型特异性验证及疾病机制探索。如需获取全文，可通过PubMed或Web of Science平台搜索标题或作者。
Calsequestrin 1 (CASQ1) is a calcium-binding protein predominantly localized in the sarcoplasmic reticulum (SR) of skeletal muscle, where it plays a critical role in regulating calcium storage and release during muscle contraction and relaxation. As a high-capacity, low-affinity calcium buffer, CASQ1 aggregates into polymeric matrices that sequester large amounts of calcium ions, ensuring efficient excitation-contraction coupling. Antibodies targeting CASQ1 are essential tools for studying its expression, distribution, and function in both physiological and pathological contexts.
CASQ1 antibodies are widely used in techniques like immunohistochemistry, Western blotting, and immunofluorescence to investigate skeletal muscle development, calcium homeostasis, and diseases such as myopathies or malignant hyperthermia. These antibodies help identify CASQ1 mutations or deficiencies linked to muscle dysfunction. Additionally, they aid in distinguishing CASQ1 from its cardiac isoform, CASQ2. which has distinct tissue specificity and disease associations.
Research involving CASQ1 antibodies has also shed light on its interactions with other SR proteins, like junctophilin and triadin, which form the calcium release complex. Dysregulation of CASQ1 is implicated in vacuolar aggregate myopathy and other neuromuscular disorders, making these antibodies valuable for diagnostic and mechanistic studies. Overall, CASQ1 antibodies serve as crucial reagents for advancing our understanding of muscle biology and calcium signaling pathologies.
×
