title: Lack of Dystrophin Affects Bronchial Epithelium in mdx Mice.
abstract: 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. J. Cell. Physiol. 231: 2218-2223, 2016. © 2016 Wiley Periodicals, Inc.
authors: Dipartimento di Biomedicina e Neuroscienze Cliniche (BioNeC), University of Palermo, Palermo, Sicilia, Italy.|Istituto di Biomedicina e Immunologia Molecolare (IBIM), Consiglio Nazionale delle Ricerche (CNR), Palermo, Sicilia, Italy.
authors: Giuseppe
authors: G
authors: Morici
authors: Dipartimento di Biomedicina e Neuroscienze Cliniche (BioNeC), University of Palermo, Palermo, Sicilia, Italy.|Dipartimento di Scienze Giuridiche della Società e dello Sport, University of Palermo, Palermo, Sicilia, Italy.|Istituto Euro-Mediterraneo di Scienza e Tecnologia, Palermo, Sicilia, Italy.
authors: Francesca
authors: F
authors: Rappa
authors: Dipartimento di Biomedicina e Neuroscienze Cliniche (BioNeC), University of Palermo, Palermo, Sicilia, Italy.|Istituto Euro-Mediterraneo di Scienza e Tecnologia, Palermo, Sicilia, Italy.
authors: Francesco
authors: F
authors: Cappello
authors: Istituto di Biomedicina e Immunologia Molecolare (IBIM), Consiglio Nazionale delle Ricerche (CNR), Palermo, Sicilia, Italy.
authors: Elisabetta
authors: E
authors: Pace
authors: Istituto Euro-Mediterraneo di Scienza e Tecnologia, Palermo, Sicilia, Italy.|Dipartimento di Scienze e Tecnologie Biologiche, Chimiche e Farmaceutiche (STEBICEF), University of Palermo, Palermo, Sicilia, Italy.
authors: Andrea
authors: A
authors: Pace
authors: Dipartimento di Biomedicina e Neuroscienze Cliniche (BioNeC), University of Palermo, Palermo, Sicilia, Italy.
authors: Giuseppa
authors: G
authors: Mudò
authors: Istituto di Biomedicina e Immunologia Molecolare (IBIM), Consiglio Nazionale delle Ricerche (CNR), Palermo, Sicilia, Italy.
authors: Grazia
authors: G
authors: Crescimanno
authors: Dipartimento di Biomedicina e Neuroscienze Cliniche (BioNeC), University of Palermo, Palermo, Sicilia, Italy.
authors: Natale
authors: N
authors: Belluardo
authors: Istituto di Biomedicina e Immunologia Molecolare (IBIM), Consiglio Nazionale delle Ricerche (CNR), Palermo, Sicilia, Italy.|Dipartimento Biomedico di Medicina Interna e Specialistica (DiBiMIS), University of Palermo, Palermo, Sicilia, Italy.
authors: Maria R
authors: MR
authors: Bonsignore
mesh_terms: D000818:Animals; D001980:Bronchi / Q000378:metabolism*; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D004848:Epithelium / Q000378:metabolism*; D015870:Gene Expression / Q000502:physiology; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D018482:Muscle, Skeletal / Q000378:metabolism*; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism*; D012038:Regeneration
chemical_list: D016189:Dystrophin