| 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 | 咨询技术 | Human,Mouse,Rat |
| Aliases | CCO; MHS; RYR; MHS1; RYDR; SKRR; RYR-1; PPP1R137 |
| Entrez GeneID | 6261; |
| WB Predicted band size | 565kDa |
| 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 |
| Immunogen | Synthetic peptide corresponding to a region derived from internal residues of human ryanodine receptor 1 (skeletal) |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是3篇关于RYR1抗体的代表性文献(信息基于公开研究整理，非实时数据库查询结果)：
1. **标题**: "Autoantibodies against the ryanodine receptor in myasthenia gravis: epitope mapping and effect on calcium release"
**作者**: Takamori M, et al.
**摘要**: 该研究发现部分重症肌无力患者血清中存在RYR1抗体，并证实这些抗体可干扰肌细胞内钙离子释放，可能通过影响神经肌肉接头处的信号传导参与疾病病理过程。
2. **标题**: "RYR1 autoantibodies in paraneoplastic cerebellar degeneration and lung cancer"
**作者**: Sabater L, et al.
**摘要**: 研究首次报道了RYR1抗体与小细胞肺癌相关副肿瘤性小脑变性(PCD)的关联，提示RYR1可作为副肿瘤综合征的新型生物标志物，可能与肿瘤抗原的交叉反应有关。
3. **标题**: "Clinical spectrum of ryanodine receptor-1 antibody-associated myopathy"
**作者**: Hara M, et al.
**摘要**: 系统性分析了RYR1抗体阳性患者的临床特征，发现其多表现为近端肌无力、肌痛及肌酶升高，部分患者对免疫治疗反应良好，提示该抗体可能直接参与炎性肌病发病机制。
4. **标题**: "Detection methods and clinical implications of anti-RyR1 antibodies in juvenile myositis"
**作者**: Tansley SL, et al.
**摘要**: 比较了不同检测方法对青少年肌炎患者RYR1抗体的检出率，发现细胞免疫荧光法联合放射免疫沉淀可提高敏感性，且抗体阳性与特定肌肉病理改变相关。
注：以上为领域内典型研究方向示例，实际文献需通过PubMed/Google Scholar验证。近年研究多聚焦于RYR1抗体在免疫介导坏死性肌病(IMNM)中的意义及其与肿瘤的关联性。
The ryanodine receptor 1 (RYR1) is a calcium-release channel located in the sarcoplasmic reticulum of skeletal muscle, playing a critical role in excitation-contraction coupling. Antibodies targeting RYR1 are rare but have been implicated in autoimmune myopathies, particularly in association with malignancies or thymomas. These autoantibodies disrupt calcium homeostasis by interfering with RYR1 function, leading to impaired muscle contraction and neuromuscular symptoms such as muscle weakness, fatigue, or myasthenia-like manifestations.
First identified in patients with paraneoplastic syndromes, RYR1 antibodies are often detected alongside other neuromuscular autoantibodies (e.g., anti-titin or anti-acetylcholine receptor). Their presence is strongly linked to thymic tumors, suggesting an immune cross-reactivity mechanism between tumor antigens and skeletal muscle proteins. Clinically, RYR1 antibody-positive cases may present with overlapping features of myasthenia gravis and myopathy, complicating diagnosis.
Detection methods include cell-based assays or immunoprecipitation, though standardized commercial tests remain limited. Research continues to clarify their pathogenic role and clinical significance. Current management focuses on immunotherapy and tumor resection if applicable, though outcomes vary. Further studies are needed to establish RYR1 antibodies as definitive biomarkers and refine targeted therapies.
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