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Endoplasmic Reticulum Stress Is Involved in Muscular Pathogenesis in Idiopathic Inflammatory Myopathies

Xue Ma1, Hua-Jie Gao1, Qing Zhang1

  • 1Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Frontiers in Cell and Developmental Biology
|March 3, 2022
PubMed

Insights

Endoplasmic reticulum (ER) stress is activated in idiopathic inflammatory myopathies (IIM), contributing to skeletal muscle damage and repair. This ER stress response correlates with disease activity in immune-mediated necrotizing myopathy (IMNM).

Area of Science:

  • Neurology
  • Immunology
  • Cell Biology

Background:

  • Endoplasmic reticulum (ER) stress is implicated in skeletal muscle damage and dysfunction across various diseases.
  • The role of ER stress in idiopathic inflammatory myopathies (IIM) remains largely unexplored.

Purpose of the Study:

  • To investigate the activation and role of ER stress in the skeletal muscle of patients with IIM.
  • To determine the correlation between ER stress markers and disease characteristics in IIM.

Main Methods:

  • Histological analysis, Western blot, and real-time quantitative Polymerase Chain Reaction were used to assess ER stress and autophagy.
  • Muscle biopsies from patients with immune-mediated necrotizing myopathy (IMNM), dermatomyositis (DM), anti-synthetase syndrome (ASS), and healthy controls were analyzed.

Main Results:

  • The ER stress-induced autophagy pathway was found to be activated in muscle biopsies from patients with IMNM, DM, and ASS.
  • Glucose-regulated protein 78 (GRP78)/BiP expression in skeletal muscle showed a correlation with autophagy, myofiber atrophy, myonecrosis, myoregeneration, and disease activity in IMNM.

Conclusions:

  • ER stress is implicated in the pathogenesis of IIM.
  • The ER stress response is linked to skeletal muscle damage and repair processes in IIM, correlating with disease activity in IMNM.
Keywords:
endoplasmic reticulum stressglucose-regulated protein 78 (GRP78)/BiPidiopathic inflammatory myopathiesimmune-mediated necrotizing myopathyskeletal muscle dysfunction

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