Increased Muscle Stress-Sensitivity Induced by Selenoprotein N Inactivation in Mouse: A Mammalian Model for SEPN1-Related Myopathy
2011

Increased Muscle Stress-Sensitivity Induced by Selenoprotein N Inactivation in Mouse

Sample size: 123 publication 10 minutes Evidence: moderate

Author Information

Author(s): Rederstorff Mathieu, Castets Perrine, Arbogast Sandrine, Lainé Jeanne, Vassilopoulos Stéphane, Beuvin Maud, Dubourg Odile, Vignaud Alban, Ferry Arnaud, Krol Alain, Allamand Valérie, Guicheney Pascale, Ferreiro Ana, Lescure Alain

Primary Institution: Architecture et Réactivité de l'ARN, Université de Strasbourg, CNRS, IBMC, Strasbourg, France

Hypothesis

What is the role of selenoprotein N in muscle function and its implications in SEPN1-Related Myopathy?

Conclusion

The study found that selenoprotein N deficiency in mice leads to increased susceptibility to muscle stress, particularly under challenging conditions.

Supporting Evidence

  • Selenoprotein N deficiency does not alter normal growth and lifespan in mice.
  • Under stress conditions, Sepn1−/− mice developed significant muscle atrophy and kyphosis.
  • The study provides a new animal model to investigate SEPN1-Related Myopathy.
  • Normal muscle function was observed in Sepn1−/− mice under standard conditions.
  • Oxidative stress levels were higher in SelN-deficient muscles after forced swimming tests.

Takeaway

Mice without a protein called selenoprotein N can swim normally, but when they are stressed, they have trouble moving and their backs curve.

Methodology

The researchers created a mouse model lacking selenoprotein N and tested their muscle response to physical stress through forced swimming tests.

Limitations

The mouse model does not fully replicate the human condition of SEPN1-Related Myopathy, as the mice do not show spontaneous muscle disorders under normal conditions.

Participant Demographics

Mice were used in the study, specifically a genetically modified strain.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1371/journal.pone.0023094

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