| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/5000 | Human,Mouse,Rat |
| 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 MUSK |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于MUSK抗体的3篇参考文献及其摘要：
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1. **文献名称**：*Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies*
**作者**：Hoch W, et al.
**摘要**：该研究首次报道了在部分血清乙酰胆碱受体(AChR)抗体阴性的重症肌无力(MG)患者中存在针对肌肉特异性激酶(MuSK)的自身抗体，揭示了MuSK抗体可能是MG的新型生物标志物，并可能与疾病病理机制直接相关。
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2. **文献名称**：*Clinical correlates with anti-MuSK antibodies in generalized seronegative myasthenia gravis*
**作者**：Evoli A, et al.
**摘要**：本研究分析了MuSK抗体阳性MG患者的临床特征，发现此类患者常表现为严重的延髓肌无力和呼吸肌受累，对传统乙酰胆碱酯酶抑制剂治疗反应不佳，但对免疫调节疗法(如利妥昔单抗)可能更敏感。
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3. **文献名称**：*MuSK antibody-positive myasthenia gravis: clinical and neurophysiological insights*
**作者**：Leite MI, et al.
**摘要**：通过比较MuSK抗体阳性与AChR抗体阳性MG患者的临床及电生理差异，研究指出MuSK阳性患者更易出现面肌和颈部肌无力，且重复神经电刺激异常率较低，提示其病理机制可能涉及突触后膜结构的特异性损伤。
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如需扩展阅读，可补充2004年*Nature Medicine*上McConville等人的研究，探讨MuSK抗体在动物模型中的致病机制。
**Background of MUSK Antibodies**
MuSK (muscle-specific tyrosine kinase) antibodies are autoantibodies associated with a subset of autoimmune myasthenia gravis (MG), a neuromuscular disorder causing muscle weakness. Discovered in 2001. MuSK-MG represents 5-8% of generalized MG cases, often affecting younger women. Unlike acetylcholine receptor (AChR) antibody-positive MG, MuSK-MG is linked to autoantibodies targeting MuSK, a key protein in neuromuscular junction (NMJ) development and maintenance.
MuSK, expressed in muscle cells, collaborates with agrin (a motor neuron-derived protein) and LRP4 to cluster AChRs during NMJ formation. Anti-MuSK IgG4 antibodies disrupt this signaling, impairing AChR organization and reducing synaptic transmission efficiency. This leads to fatigable weakness, predominantly in facial, bulbar, and respiratory muscles, with symptoms like ptosis, dysphagia, and respiratory crises.
Clinically, MuSK-MG is often severe, with poor response to standard acetylcholinesterase inhibitors (e.g., pyridostigmine). Diagnosis relies on serum antibody testing, distinguishing it from AChR-MG. Treatments include immunosuppressants (e.g., corticosteroids, rituximab) and plasmapheresis. Research continues to unravel MuSK's role in autoimmunity, aiming to refine targeted therapies for this distinct MG subtype.
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