Animal models of antimuscle-specific kinase myasthenia.
Animal models of antimuscle-specific kinase myasthenia.
复制标题
抗肌肉特异性激酶肌无力的动物模型。
DOI：
10.1111/j.1749-6632.2012.06782.x
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发表时间：
2012
影响因子：
5.2
通讯作者：
Agius,MarkA
中科院分区：
文献类型：
--
作者：
Richman,DavidP;Nishi,Kayoko;Ferns,MichaelJ;Schnier,Joachim;Pytel,Peter;Maselli,RicardoA;Agius,MarkA
抗肌肉特异性激酶（抗MuSK）肌无力（AMM）与抗乙酰胆碱受体重症肌无力不同，其表现为更多局灶性肌肉受累（颈、肩、面部和延髓肌肉），受累肌肉（主要是轴向肌肉）消瘦。AMM与胸腺增生无关，对抗胆碱酯酶治疗反应差。通过用纯化的异种MuSK胞外域免疫家兔、小鼠和大鼠，并通过将大量纯化的AMM患者血清IgG被动转移到小鼠中，已经在家兔、小鼠和大鼠中诱导了AMM动物模型。该模型证实了MuSK抗体在AMM中的致病作用，并证明了神经肌肉接头的突触前和突触后成分的参与。在这种人类疾病及其动物模型中的观察表明，MuSK不仅在这种突触的形成中，而且在其维持中发挥作用。
Antimuscle‐specific kinase (anti‐MuSK) myasthenia (AMM) differs from antiacetylcholine receptor myasthenia gravis in exhibiting more focal muscle involvement (neck, shoulder, facial, and bulbar muscles) with wasting of the involved, primarily axial, muscles. AMM is not associated with thymic hyperplasia and responds poorly to anticholinesterase treatment. Animal models of AMM have been induced in rabbits, mice, and rats by immunization with purified xenogeneic MuSK ectodomain, and by passive transfer of large quantities of purified serum IgG from AMM patients into mice. The models have confirmed the pathogenic role of the MuSK antibodies in AMM and have demonstrated the involvement of both the presynaptic and postsynaptic components of the neuromuscular junction. The observations in this human disease and its animal models demonstrate the role of MuSK not only in the formation of this synapse but also in its maintenance.