Lack of Dystrophin Affects Bronchial Epithelium in mdx Mice

Giuseppe Morici, Giuseppa Mudo', Francesca Rappa, Andrea Pace, Maria Rosaria Bonsignore, Francesco Cappello, Natale Belluardo, Grazia Crescimanno, Andrea Pace, Maria R. Bonsignore, Grazia Crescimanno, Francesca Rappa, Giuseppe Morici, Elisabetta Pace, Francesco Cappello

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2 Citations (Scopus)


Mild exercise training may positively affect the course of Duchenne Muscular Dystrophy (DMD). Training causes mild bronchial epithelial injury in both humans and mice, but no study assessed the effects of exercise in mdx mice, a well known model of DMD. The airway epithelium was examined in mdx (C57BL/10ScSn-Dmdmdx) mice, and in wild type (WT, C57BL/10ScSc) mice either under sedentary conditions (mdx-SD, WT-SD) or during mild exercise training (mdx-EX, WT-EX). At baseline, and after 30 and 45 days of training (5 d/wk for 6 weeks), epithelial morphology and markers of regeneration, apoptosis, and cellular stress were assessed. The number of goblet cells in bronchial epithelium was much lower in mdx than in WT mice under all conditions. At 30 days, epithelial regeneration (PCNA positive cells) was higher in EX than SD animals in both groups; however, at 45 days, epithelial regeneration decreased in mdx mice irrespective of training, and the percentage of apoptotic (TUNEL positive) cells was higher in mdx-EX than in WT-EX mice. Epithelial expression of HSP60 (marker of stress) progressively decreased, and inversely correlated with epithelial apoptosis (r=-0.66, P=0.01) only in mdx mice. Lack of dystrophin in mdx mice appears associated with defective epithelial differentiation, and transient epithelial regeneration during mild exercise training. Hence, lack of dystrophin might impair repair in bronchial epithelium, with potential clinical consequences in DMD patients.
Original languageEnglish
Pages (from-to)2218-2223
Number of pages6
JournalJournal of Cellular Physiology
Publication statusPublished - 2016

All Science Journal Classification (ASJC) codes

  • Physiology
  • Clinical Biochemistry
  • Cell Biology

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