骨骼肌束周萎缩是皮肌炎特征性病理改变，IFNβ的产生是引起束周萎缩重要因素，但调控其产生的机制不清楚。我们前期证实，骨骼肌高表达的新的lincRNA-p33629、及其预测的靶基因干扰素诱导基因USP18与皮肌炎束周萎缩高度相关，但尚不清楚lincRNA是否参与调控IFN-β形成继而引起束周萎缩。因此，本项目以皮肌炎患者骨骼肌细胞入手，采用免疫与分子生物等技术，围绕linc-RNA-USP18-IFNβ-骨骼肌损伤，探讨：1）lincRNA-p33629及USP18对皮肌炎骨骼肌细胞萎缩的影响。2）linRNA-p33629及USP18诱导骨骼肌细胞产生IFNβ的过程。3）lincRNA靶向调控USP18诱导皮肌炎骨骼肌细胞产生IFNβ形成及其机制。本项目将系统研究lincRNA调控IFNβ产生在皮肌炎骨骼肌束周萎缩的分子机制，为皮肌炎的诊断及寻求特异性干预靶点提供新的思路。

Skeletal muscle perifascicular atrophy is unique pathological change of dermatomyositis, IFN beta is key regulator in perifascicular atrophy of dermatomyositis, but the regulation of its production mechanism is still unknown. Our previous results showed high expression of novel lincRNA-p33629 in skeletal muscle , and its predicted target gene interferon inducible gene USP18 were both closely associated with dermatomyositis perifascicular atrophy. However, it is unclear that whether lincRNA could participate in the process of muscle perifascicular atrophy triggerd by IFN- beta production. Therefore, this study will focus on the linc-RNA-USP18-IFN beta-skeletal muscle damage in dermatomyositis, we aim to explore: 1) effects of lincRNA-p33629 and USP18 on skeletal muscle cell atrophy in dermatomyositis. 2) linRNA-p33629 and USP18 on the production of IFN beta in skeletal muscle cells from dermatomyositis. 3) lincRNA promote the production of IFN beta from skeletal muscle cells by targeting regulation of USP18 in dermatomyositis. This project will systematicly study on the molecular mechanism of lincRNA promote skeletal muscle atrophy triggerd with IFN beta in dermatomyositis, which could provide a new way for the diagnosis of dermatomyositis and seek specific targets for intervention.