Effects of the selective inhibition of proteasome caspase-like activity by CLi a derivative of nor-cerpegin in dystrophic mdx mice

PLoS One. 2019 Apr 23;14(4):e0215821. doi: 10.1371/journal.pone.0215821. eCollection 2019.

Abstract

Previous studies have shown that proteasome inhibition can have beneficial effects in dystrophic mouse models. In this study, we have investigated the effects of a new selective proteasome inhibitor, CLi, a strong caspase-like inhibitor of the 20S proteasome, on skeletal and cardiac muscle functions of mdx mice. In the first series of experiments, five-month-old male mdx mice (n = 34) were treated with 2 different doses (20 and 100 μg/kg) of CLi and in the second series of experiments, five-month-old female mdx (n = 19) and wild-type (n = 24) mice were treated with 20 μg/kg CLi and Velcade (1 mg/kg) for 1-month. All animals were treadmill exercised twice a week to worsen the dystrophic features. In the first series of experiments, our results demonstrated that 20 μg/kg CLi did not significantly increase absolute and specific maximal forces in skeletal muscle from male mdx mice. Moreover, the higher susceptibility to contraction induced skeletal muscle injury was worsened by 100 μg/kg CLi since the force drop following lengthening contractions was increased with this high dose. Furthermore, we found no differences in the mRNA levels of the molecular markers implicated in dystrophic features. Concerning cardiac function, CLi had no effect on left ventricular function since ejection and shortening fractions were unchanged in male mdx mice. Similarly, CLi did not modify the expression of genes implicated in cardiac remodeling. In the second series of experiments, our results demonstrated an improvement in absolute and specific maximal forces by CLi, whereas Velcade only increased specific maximal force in female mdx mice. In addition, exercise tolerance was not improved by CLi. Taken together, our results show that CLi treatment can only improve maximal force production in exercised female mdx mice without affecting either exercice tolerance capacity or cardiac function. In conclusion, selective inhibition of caspase-like activity of proteasome with CLi has no compelling beneficial effect in dystrophic mdx mice.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Caspase Inhibitors / chemistry
  • Caspase Inhibitors / pharmacology*
  • Caspases / genetics
  • Disease Models, Animal
  • Dystrophin / genetics
  • Female
  • Heart / drug effects
  • Male
  • Mice
  • Mice, Inbred mdx
  • Muscle Contraction / drug effects*
  • Muscle, Skeletal / drug effects
  • Physical Conditioning, Animal
  • Proteasome Endopeptidase Complex / drug effects*
  • Proteasome Endopeptidase Complex / genetics
  • Proteasome Inhibitors / chemistry
  • Proteasome Inhibitors / pharmacology*
  • Pyridones / chemistry
  • Pyridones / pharmacology*

Substances

  • Caspase Inhibitors
  • Dystrophin
  • Proteasome Inhibitors
  • Pyridones
  • cerpegin
  • Caspases
  • Proteasome Endopeptidase Complex

Grants and funding

This work was supported by funds from CNRS (OA), Sorbonne University (OA), University Paris Descartes (AF), the Association Française contre les Myopathies (OA, contract number:16605), the LabEx REVIVE (OA, ANR-10-LABX-73). Y.H. supported by a fellowship from the Association Française contre les Myopathies. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.