Day Jessica, Otto Sophia, Cash Kathy, Eldi Preethi, Hissaria Pravin, Proudman Susanna, Limaye Vidya, Hayball John D
Experimental Therapeutics Laboratory, University of South Australia Cancer Research Institute, Adelaide, SA, Australia.
School of Pharmacy and Medical Sciences, University of South Australia, Adelaide, SA, Australia.
Front Cell Dev Biol. 2020 Apr 17;8:226. doi: 10.3389/fcell.2020.00226. eCollection 2020.
High Mobility Group Box Protein 1 (HMGB1) is a DNA-binding protein that exerts inflammatory or pro-repair effects upon translocation from the nucleus. We postulate aberrant HMGB1 expression in immune-mediated necrotising myopathy (IMNM).
Herein, we compare HMGB1 expression (serological and sarcoplasmic) in patients with IMNM with that of other myositis subtypes using immunohistochemistry and ELISA.
IMNM ( = 62) and inclusion body myositis (IBM, = 14) patients had increased sarcoplasmic HMGB1 compared with other myositis patients ( = 46). Sarcoplasmic HMGB1 expression correlated with muscle weakness and histological myonecrosis, inflammation, regeneration and autophagy. Serum HMGB1 levels were elevated in patients with IMNM, dermatomyositis and polymositis, and those myositis patients with extramuscular inflammatory features.
Aberrant HMGB1 expression occurs in myositis patients and correlates with weakness. A unique expression profile of elevated sarcoplasmic and serum HMGB1 was detected in IMNM.
高迁移率族蛋白B1(HMGB1)是一种DNA结合蛋白,从细胞核易位后可发挥炎症或促修复作用。我们推测免疫介导的坏死性肌病(IMNM)中存在HMGB1表达异常。
在此,我们使用免疫组织化学和酶联免疫吸附测定法(ELISA)比较IMNM患者与其他肌炎亚型患者的HMGB1表达(血清学和肌浆内)情况。
与其他肌炎患者(n = 46)相比,IMNM患者(n = 62)和包涵体肌炎(IBM,n = 14)患者的肌浆内HMGB1增加。肌浆内HMGB1表达与肌肉无力以及组织学上的肌坏死、炎症、再生和自噬相关。IMNM、皮肌炎和多发性肌炎患者以及那些具有肌肉外炎症特征的肌炎患者血清HMGB1水平升高。
肌炎患者中存在HMGB1表达异常,且与肌无力相关。在IMNM中检测到肌浆内和血清HMGB1升高的独特表达谱。