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Published on: August 9, 2024
Selenoprotein N in skeletal muscle: from diseases to function
Perrine Castets1, Alain Lescure, Pascale Guicheney
1Neuromuscular Centre, University Hospital of Basel, 4056, Basel, Switzerland.
Journal of Molecular Medicine (Berlin, Germany)
|April 25, 2012
Summary
Selenoprotein N (SelN) deficiency causes SEPN1-related myopathies. This review highlights SelN
Area of Science:
- Biochemistry
- Molecular Biology
- Genetics
Background:
- SEPN1-related myopathies result from Selenoprotein N (SelN) deficiency, causing progressive muscle weakness and respiratory issues.
- SelN's precise function in muscle tissue and its role in disease pathogenesis remain largely undetermined.
Purpose of the Study:
- To review the expression patterns and known functions of SelN across species (zebrafish, mice, humans).
- To explore SelN's potential roles in myogenesis, muscle regeneration, and cellular homeostasis.
- To discuss emerging therapeutic avenues for SEPN1-related myopathies.
Main Methods:
- Review of existing literature on SelN expression, function, and related myopathies.
- Analysis of data from zebrafish, mouse, and human studies.
Main Results:
- SelN is crucial for muscle regeneration and satellite cell maintenance in mice and humans, contrary to earlier findings.
- Emerging evidence suggests SelN involvement in oxidative and calcium homeostasis, potentially regulating ryanodine receptor activity.
Conclusions:
- SelN plays a vital role in muscle health beyond development, particularly in regeneration and maintenance.
- Understanding SelN's cellular functions offers new insights into SEPN1-related myopathies and potential therapeutic strategies.
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