ER stress and ERO1: Potential therapeutic targets for inherited myopathies

Cell Rep Med. 2024 Mar 19;5(3):101462. doi: 10.1016/j.xcrm.2024.101462.

Abstract

Selenoprotein N-related myopathy (SEPN1-RM) is a genetic disease that causes muscle weakness and respiratory failure. Germani et al.1 demonstrate that diaphragm weakness in SEPN1-RM is prevented by the inhibition of ER stress or ERO1 oxidoreductase regulated by transcription factor CHOP.

MeSH terms

  • Humans
  • Muscle Proteins / genetics
  • Muscular Diseases* / genetics
  • Muscular Diseases* / therapy
  • Oxidative Stress / genetics
  • Respiratory Insufficiency*
  • Selenoproteins / genetics
  • Selenoproteins / metabolism

Substances

  • Muscle Proteins
  • Selenoproteins