肌肉活检中ER诱导自噬标志物的表达水平与特发性炎症性肌病病情的相关性
Correlation between the expression of ER-induced autophagy markers in muscle biopsy and idiopathic inflam-matory myopathy
孙广珍 1王慧 1李阳1
作者信息
- 1. 150000 哈尔滨,北大荒集团总医院(黑龙江省第二肿瘤医院)神经内科
- 折叠
摘要
目的 探讨肌肉活检中ER诱导自噬标志物的表达水平与特发性炎症性肌病(Idiopathic in-flammatory myopathy,IIM)病情的相关性.方法 37例免疫介导坏死性肌病(Immune-mediated necrotizing myopathy,IMNM)患者、14 例皮肌炎(Dermatomyositis,DM)患者、6 例抗合成酶综合征(Antisynthase syn-drome,ASS)患者和10例对照者纳入研究;采用组织学切片、Western blot和实时定量聚合酶链式反应(Poly-merase chain reaction,PCR)检测内质网应激诱导自噬通路的表达水平.结果 内质网应激诱导的自噬途径在IMNM,DM和ASS患者的活检肌肉中被激活,骨骼肌中内质网伴侣蛋白、葡萄糖调节蛋白78(Glucose-regulating protein 78,GRP78)/免疫球蛋白结合蛋白(Immunoglobulin binding protein,BiP)表达水平与 IM-NM的自噬、肌纤维萎缩、肌坏死、肌再生和疾病活动性相关.结论 内质网应激自噬与特发性炎症性肌病患者有关,并与IMNM的疾病活动性相关,内质网应激反应可能是IIM患者骨骼肌损伤和修复的原因.
Abstract
Objective To investigate the correlation between the expression of ER-induced autophagy markers in muscle biopsy and idiopathic inflammatory myopathy(IIM).Methods Thirty-seven patients with immune-mediated necrotizing myopathy(IMNM),14 patients with dermatomyositis(DM),6 patients with anti-synthase syndrome(ASS),and 10 controls were included in the study.Histological sections,Western blot and real-time quantitative PCR were used to detect the expression of endoplasmic reticulum stress-induced autophagy pathway.Results Endoplasmic reticulum stress-induced autophagy pathway is activated in biopsied muscles of patients with IMNM,DM,and ASS,and the expression of endoplasmic reticulin,glucose-regulato-ry protein 78(GRP78)/BiP in skeletal muscle is associated with IMNM autophagy,muscle fiber atrophy,muscle necrosis,muscle regeneration,and disease activity.Conclusion Endoplasmic reticulum stress autoph-agy is associated with IIM patients and disease activity of IMNM,and the endoplasmic reticulum stress re-sponse may be the cause of skeletal muscle injury and repair in IIM.
关键词
内质网应激/自噬/肌张力性/骨骼肌Key words
Endoplasmic reticulum stress/Autophagy/Muscular tone/Skeletal muscle引用本文复制引用
基金项目
黑龙江省卫生健康委员会科研项目(2021WJ0561)
出版年
2024
NETL
NSTL
万方数据