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首页> 外文期刊>Journal of Neuroimmunology: Official Bulletin of the Research Committee on Neuroimmunology of the World Federation of Neurology >Experimental allergic myositis in SJL/J mouse. Reappraisal of immune reaction based on changes after single immunization.
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Experimental allergic myositis in SJL/J mouse. Reappraisal of immune reaction based on changes after single immunization.

机译：SJL / J小鼠的实验性过敏性肌炎。根据单次免疫后的变化重新评估免疫反应。

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SJL/J mice have been subjected to immunization with wide varieties of antigens to produce models of autoimmune disorders including experimental myositis. They also have a defect in dysferlin gene and spontaneously develop muscle fiber degeneration, a condition akin to limb-girdle type muscular dystrophy and Miyoshi myopathy. To know whether muscle inflammation of SJL mice after immunization with muscle fractions really represents immune-mediated myositis or no more than an epiphenomenon of muscle degeneration due to dysferlin defect, we studied immunological parameters after immunization with rabbit myosin B fraction. Initial infiltration of macrophages and CD4+ lymphocytes on day 11 was followed by increase in number of CD8+ cells. Such increase was not observed in the nontreated and adjuvant controls. Some infiltrating cells were interferon gamma (IFN-gamma) positive. Furthermore, increased expression of the signal transducers and activator of transcription 1 (STAT-1) and interferon regulatory factor 1 (IRF-1) mRNA was shown in the first 2 weeks. These results indicate Th1 system activity in the muscle, rather than simple dysferlin deficiency, particularly 1-3 weeks after immunization. Thus it is concluded that an immune-mediated myositis is taking place at this stage. This model can be helpful in understanding pathomechanisms involved in the early stage of human myositides. It has also important implications concerning immune reactions associated with transplantation or gene therapy for muscular dystrophies.
机译：SJL / J小鼠已经接受了多种抗原的免疫接种，以产生包括实验性肌炎在内的自身免疫疾病模型。它们在dysferlin基因上也有缺陷，并自发发展为肌纤维变性，类似于肢带型肌肉营养不良和三好肌病。要知道用肌肉部分免疫后SJL小鼠的肌肉炎症是否确实代表了免疫介导的肌炎，还是只不过是由dysferlin缺陷引起的肌肉变性的表象，我们研究了兔肌球蛋白B部分免疫后的免疫学参数。在第11天，巨噬细胞和CD4 +淋巴细胞开始浸润，随后CD8 +细胞数目增加。在未治疗和佐剂对照中未观察到这种增加。一些浸润细胞是干扰素γ（IFN-γ）阳性。此外，在最初的两周内，信号转导和转录激活因子1（STAT-1）和干扰素调节因子1（IRF-1）mRNA的表达增加。这些结果表明Th1系统在肌肉中的活动，而不是简单的dysferlin缺乏，特别是在免疫后1-3周。因此可以得出结论，在这一阶段正在发生免疫介导的肌炎。该模型有助于理解人类肌肽早期所涉及的病理机制。对于与肌营养不良的移植或基因治疗有关的免疫反应，它也具有重要意义。

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