{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T19:09:43Z","timestamp":1790622583832,"version":"4.1.0"},"reference-count":83,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2019,11,24]],"date-time":"2019-11-24T00:00:00Z","timestamp":1574553600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"The Physiological Society","award":["Research Grant to DE"],"award-info":[{"award-number":["Research Grant to DE"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antioxidants"],"abstract":"<jats:p>Respiratory muscle weakness occurs due to dystrophin deficiency in Duchenne muscular dystrophy (DMD). The mdx mouse model of DMD shows evidence of impaired respiratory muscle performance with attendant inflammation and oxidative stress. We examined the effects of N-acetylcysteine (NAC) supplementation on respiratory system performance in mdx mice. Eight-week-old male wild type (n = 10) and mdx (n = 20) mice were studied; a subset of mdx (n = 10) received 1% NAC in the drinking water for 14 days. We assessed breathing, diaphragm, and external intercostal electromyogram (EMG) activities and inspiratory pressure during ventilatory and non-ventilatory behaviours. Diaphragm muscle structure and function, cytokine concentrations, glutathione status, and mRNA expression were determined. Diaphragm force-generating capacity was impaired in mdx compared with wild type. Diaphragm muscle remodelling was observed in mdx, characterized by increased muscle fibrosis, immune cell infiltration, and central myonucleation. NAC supplementation rescued mdx diaphragm function. Collagen content and immune cell infiltration were decreased in mdx + NAC compared with mdx diaphragms. The cytokines IL-1\u03b2, IL-6 and KC\/GRO were increased in mdx plasma and diaphragm compared with wild type; NAC decreased systemic IL-1\u03b2 and KC\/GRO concentrations in mdx mice. We reveal that NAC treatment improved mdx diaphragm force-generating capacity associated with beneficial anti-inflammatory and anti-fibrotic effects. These data support the potential use of NAC as an adjunctive therapy in human dystrophinopathies.<\/jats:p>","DOI":"10.3390\/antiox8120581","type":"journal-article","created":{"date-parts":[[2019,11,25]],"date-time":"2019-11-25T03:10:00Z","timestamp":1574651400000},"page":"581","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["N-acetylcysteine Decreases Fibrosis and Increases Force-Generating Capacity of mdx Diaphragm"],"prefix":"10.3390","volume":"8","author":[{"given":"David P.","family":"Burns","sequence":"first","affiliation":[{"name":"Department of Physiology, School of Medicine, College of Medicine and Health, University College Cork, T12 XF62 Cork, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah E.","family":"Drummond","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, College of Medicine and Health, University College Cork, T12 XF62 Cork, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dearbhla","family":"Bolger","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, Trinity Biomedical Sciences Institute, Trinity College Dublin, The University of Dublin, D02 R590 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Am\u00e9lie","family":"Coiscaud","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, College of Medicine and Health, University College Cork, T12 XF62 Cork, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin H.","family":"Murphy","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, College of Medicine and Health, University College Cork, T12 XF62 Cork, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deirdre","family":"Edge","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, Trinity Biomedical Sciences Institute, Trinity College Dublin, The University of Dublin, D02 R590 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken D.","family":"O\u2019Halloran","sequence":"additional","affiliation":[{"name":"Department of Physiology, School of Medicine, College of Medicine and Health, University College Cork, T12 XF62 Cork, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,11,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"236","DOI":"10.4103\/0028-3886.43441","article-title":"Duchenne muscular dystrophy","volume":"56","author":"Yiu","year":"2008","journal-title":"Neurol. India"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3710","DOI":"10.1073\/pnas.90.8.3710","article-title":"Dystrophin protects the sarcolemma from stresses developed during muscle contraction","volume":"90","author":"Petrof","year":"1993","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1016\/0092-8674(91)90035-W","article-title":"Membrane organization of the dystrophin-glycoprotein complex","volume":"66","author":"Ervasti","year":"1991","journal-title":"Cell"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2781","DOI":"10.1093\/hmg\/ddx173","article-title":"Oxidative stress in Duchenne muscular dystrophy: focus on the NRF2 redox pathway","volume":"26","author":"Petrillo","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.pediatrneurol.2006.09.016","article-title":"Pathophysiology of duchenne muscular dystrophy: current hypotheses","volume":"36","author":"Deconinck","year":"2007","journal-title":"Pediatr. Neurol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1002\/ppul.22847","article-title":"Respiratory muscle decline in Duchenne muscular dystrophy","volume":"49","author":"Khirani","year":"2014","journal-title":"Pediatr. Pulmonol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1002\/ppul.23172","article-title":"Characterization of pulmonary function in Duchenne Muscular Dystrophy","volume":"50","author":"Mayer","year":"2015","journal-title":"Pediatr. Pulmonol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1016\/j.nmd.2016.12.014","article-title":"Characterization of pulmonary function in 10-18 year old patients with Duchenne muscular dystrophy","volume":"27","author":"Meier","year":"2017","journal-title":"Neuromuscul. Disord."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1136\/thx.52.5.472","article-title":"Diaphragm thickness and inspiratory strength in patients with Duchenne muscular dystrophy","volume":"52","author":"Ueki","year":"1997","journal-title":"Thorax"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1403","DOI":"10.1183\/09031936.94.07081403","article-title":"Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy","volume":"7","author":"McCann","year":"1994","journal-title":"Eur. Respir. J."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2052","DOI":"10.3109\/09638288.2015.1111434","article-title":"Dysphagia in Duchenne muscular dystrophy: practical recommendations to guide management","volume":"38","author":"Toussaint","year":"2016","journal-title":"Disabil. Rehabil."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"6653","DOI":"10.1113\/JP274792","article-title":"Sensorimotor control of breathing in the mdx mouse model of Duchenne muscular dystrophy","volume":"595","author":"Burns","year":"2017","journal-title":"J. Physiol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1113\/JP277443","article-title":"Inspiratory pressure-generating capacity is preserved during ventilatory and non-ventilatory behaviours in young dystrophic mdx mice despite profound diaphragm muscle weakness","volume":"597","author":"Burns","year":"2019","journal-title":"J. Physiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1744","DOI":"10.1152\/japplphysiol.00989.2002","article-title":"Severe muscle dysfunction precedes collagen tissue proliferation in mdx mouse diaphragm","volume":"94","author":"Coirault","year":"2003","journal-title":"J. Appl. Physiol. (1985)"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"C1170","DOI":"10.1152\/ajpcell.1999.277.6.C1170","article-title":"Myosin molecular motor dysfunction in dystrophic mouse diaphragm","volume":"277","author":"Coirault","year":"1999","journal-title":"Am. J. Physiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1038\/352536a0","article-title":"The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy","volume":"352","author":"Stedman","year":"1991","journal-title":"Nature"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"5175","DOI":"10.1113\/JP276954","article-title":"Recovery of respiratory function in mdx mice co-treated with neutralizing interleukin-6 receptor antibodies and urocortin-2","volume":"596","author":"Burns","year":"2018","journal-title":"J. Physiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1007\/s12011-015-0290-y","article-title":"Reduction of Oxidative Damage and Inflammatory Response in the Diaphragm Muscle of mdx Mice Using Iron Chelator Deferoxamine","volume":"167","author":"Moraes","year":"2015","journal-title":"Biol. Trace Elem. Res."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1152\/jappl.2001.90.2.724","article-title":"Invited Review: redox modulation of skeletal muscle contraction: what we know and what we don\u2019t","volume":"90","author":"Reid","year":"2001","journal-title":"J. Appl. Physiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"75","DOI":"10.2147\/HP.S141283","article-title":"Respiratory muscle dysfunction in animal models of hypoxic disease: antioxidant therapy goes from strength to strength","volume":"5","author":"Lewis","year":"2017","journal-title":"Hypoxia (Auckl)"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2161","DOI":"10.1113\/jphysiol.2011.207456","article-title":"Exacerbation of pathology by oxidative stress in respiratory and locomotor muscles with Duchenne muscular dystrophy","volume":"589","author":"Lawler","year":"2011","journal-title":"J. Physiol."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Burns, D.P., Ali, I., Rieux, C., Healy, J., Jasionek, G., and O\u2019Halloran, K.D. (2017). Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice. Antioxidants, 6.","DOI":"10.3390\/antiox6040101"},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Hermes, T.A., M\u00e2ncio, R.D., Macedo, A.B., Mizobuti, D.S., Rocha, G.L.D., Cagnon, V.H.A., and Minatel, E. (2019). Tempol treatment shows phenotype improvement in mdx mice. PLoS ONE, 14.","DOI":"10.1371\/journal.pone.0215590"},{"key":"ref_24","first-page":"4805493","article-title":"Diaphragm Muscle Weakness Following Acute Sustained Hypoxic Stress in the Mouse Is Prevented by Pretreatment with N-Acetyl Cysteine","volume":"2018","author":"Drummond","year":"2018","journal-title":"Oxid. Med. Cell Longev."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1165\/rcmb.2015-0272OC","article-title":"Redox Remodeling Is Pivotal in Murine Diaphragm Muscle Adaptation to Chronic Sustained Hypoxia","volume":"55","author":"Lewis","year":"2016","journal-title":"Am. J. Respir. Cell. Mol. Biol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1113\/expphysiol.2013.076828","article-title":"Reactive oxygen species mediated diaphragm fatigue in a rat model of chronic intermittent hypoxia","volume":"99","author":"Shortt","year":"2014","journal-title":"Exp. Physiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"7093","DOI":"10.1113\/JP274229","article-title":"Pre-clinical evaluation of N-acetylcysteine reveals side effects in the mdx mouse model of Duchenne muscular dystrophy","volume":"595","author":"Pinniger","year":"2017","journal-title":"J. Physiol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/j.clnu.2012.06.001","article-title":"N-acetylcysteine treatment reduces TNF-\u03b1 levels and myonecrosis in diaphragm muscle of mdx mice","volume":"32","author":"Ferretti","year":"2013","journal-title":"Clin. Nutr."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/j.nmd.2011.11.007","article-title":"N-Acetylcysteine treatment of dystrophic mdx mice results in protein thiol modifications and inhibition of exercise induced myofibre necrosis","volume":"22","author":"Terrill","year":"2012","journal-title":"Neuromuscul. Disord."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"2003","DOI":"10.1113\/jphysiol.2007.148338","article-title":"N-Acetylcysteine ameliorates skeletal muscle pathophysiology in mdx mice","volume":"586","author":"Whitehead","year":"2008","journal-title":"J. Physiol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2547","DOI":"10.1113\/JP270818","article-title":"Principles and standards for reporting animal experiments in The Journal of Physiology and Experimental Physiology","volume":"593","author":"Grundy","year":"2015","journal-title":"J. Physiol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.resp.2015.12.001","article-title":"Evidence of hypoxic tolerance in weak upper airway muscle from young mdx mice","volume":"226","author":"Burns","year":"2016","journal-title":"Respir. Physiol. Neurobiol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1113\/EP086232","article-title":"Restoration of pharyngeal dilator muscle force in dystrophin-deficient (mdx) mice following co-treatment with neutralizing interleukin-6 receptor antibodies and urocortin 2","volume":"102","author":"Burns","year":"2017","journal-title":"Exp. Physiol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"122","DOI":"10.3389\/fphys.2015.00122","article-title":"Chronic sustained hypoxia-induced redox remodeling causes contractile dysfunction in mouse sternohyoid muscle","volume":"6","author":"Lewis","year":"2015","journal-title":"Front. Physiol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/s13395-015-0046-6","article-title":"Developmental myosins: expression patterns and functional significance","volume":"5","author":"Schiaffino","year":"2015","journal-title":"Skelet Muscle"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s10974-005-9014-9","article-title":"TGF-beta1 favors the development of fast type identity during soleus muscle regeneration","volume":"27","author":"Noirez","year":"2006","journal-title":"J. Muscle Res. Cell Motil."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1007\/s11910-017-0750-1","article-title":"Sleep Disordered Breathing in Duchenne Muscular Dystrophy","volume":"17","author":"LoMauro","year":"2017","journal-title":"Curr. Neurol. Neurosci. Rep."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1346","DOI":"10.1378\/chest.96.6.1346","article-title":"Ventilation and breathing pattern during sleep in Duchenne muscular dystrophy","volume":"96","author":"Smith","year":"1989","journal-title":"Chest"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1002\/(SICI)1099-0496(200002)29:2<135::AID-PPUL8>3.0.CO;2-#","article-title":"Sleep-disordered breathing in Duchenne muscular dystrophy: a preliminary study of the role of portable monitoring","volume":"29","author":"Kirk","year":"2000","journal-title":"Pediatr. Pulmonol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1136\/thx.49.2.157","article-title":"Obstructive apnoeas in Duchenne muscular dystrophy","volume":"49","author":"Khan","year":"1994","journal-title":"Thorax"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1111\/j.1440-1754.2005.00691.x","article-title":"Sleep-related breathing disorder in Duchenne muscular dystrophy: disease spectrum in the paediatric population","volume":"41","author":"Suresh","year":"2005","journal-title":"J. Paediatr. Child Health"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1007\/978-3-319-18440-1_27","article-title":"Respiratory Control in the mdx Mouse Model of Duchenne Muscular Dystrophy","volume":"860","author":"Burns","year":"2015","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Mosqueira, M., Baby, S.M., Lahiri, S., and Khurana, T.S. (2013). Ventilatory chemosensory drive is blunted in the mdx mouse model of Duchenne Muscular Dystrophy (DMD). PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0069567"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s10974-007-9101-1","article-title":"Ventilation during air breathing and in response to hypercapnia in 5 and 16 month-old mdx and C57 mice","volume":"28","author":"Gayraud","year":"2007","journal-title":"J. Muscle Res. Cell Motil."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1164\/ajrccm.162.1.9905063-r2","article-title":"Severe mechanical dysfunction in pharyngeal muscle from adult mdx mice","volume":"162","author":"Attal","year":"2000","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_46","first-page":"1553","article-title":"Mechanical properties of respiratory muscles","volume":"3","author":"Sieck","year":"2013","journal-title":"Compr. Physiol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1016\/B978-0-444-63274-6.00016-3","article-title":"Convergence of pattern generator outputs on a common mechanism of diaphragm motor unit recruitment","volume":"209","author":"Mantilla","year":"2014","journal-title":"Prog. Brain Res."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.resp.2018.06.009","article-title":"Breathing with neuromuscular disease: Does compensatory plasticity in the motor drive to breathe offer a potential therapeutic target in muscular dystrophy?","volume":"265","author":"Burns","year":"2019","journal-title":"Respir Physiol Neurobiol"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1111\/iep.12142","article-title":"Changes in calsequestrin, TNF-\u03b1, TGF-\u03b2 and MyoD levels during the progression of skeletal muscle dystrophy in mdx mice: a comparative analysis of the quadriceps, diaphragm and intrinsic laryngeal muscles","volume":"96","author":"Ferretti","year":"2015","journal-title":"Int. J. Exp. Pathol."},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Gutpell, K.M., Hrinivich, W.T., and Hoffman, L.M. (2015). Skeletal muscle fibrosis in the mdx\/utrn+\/- mouse validates its suitability as a murine model of Duchenne muscular dystrophy. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0117306"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1096\/fj.06-7285com","article-title":"Gene expression profiling in the early phases of DMD: a constant molecular signature characterizes DMD muscle from early postnatal life throughout disease progression","volume":"21","author":"Pescatori","year":"2007","journal-title":"FASEB J."},{"key":"ref_52","first-page":"917","article-title":"Upregulation of chemokines and their receptors in Duchenne muscular dystrophy: potential for attenuation of myofiber necrosis","volume":"46","author":"Creus","year":"2012","journal-title":"Muscle Nerve"},{"key":"ref_53","first-page":"16","article-title":"Activation of NF-kappaB pathway in Duchenne muscular dystrophy: relation to age","volume":"30","author":"Messina","year":"2011","journal-title":"Acta Myol."},{"key":"ref_54","first-page":"891972","article-title":"Systemic Inflammation in Duchenne Muscular Dystrophy: Association with Muscle Function and Nutritional Status","volume":"2015","year":"2015","journal-title":"Biomed. Res. Int."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"917","DOI":"10.2119\/molmed.2010.00256","article-title":"Rapamycin ameliorates dystrophic phenotype in mdx mouse skeletal muscle","volume":"17","author":"Eghtesad","year":"2011","journal-title":"Mol. Med."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1002\/mus.23432","article-title":"Inflammation in muscular dystrophy and the beneficial effects of non-steroidal anti-inflammatory drugs","volume":"46","author":"Serra","year":"2012","journal-title":"Muscle Nerve"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.ebiom.2015.02.014","article-title":"Functional and Morphological Improvement of Dystrophic Muscle by Interleukin 6 Receptor Blockade","volume":"2","author":"Pelosi","year":"2015","journal-title":"EBioMedicine"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"E134","DOI":"10.1002\/mus.25644","article-title":"Combined xIL-6R and urocortin-2 treatment restores mdx diaphragm muscle force","volume":"56","author":"Manning","year":"2017","journal-title":"Muscle Nerve"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1987218","DOI":"10.1155\/2017\/1987218","article-title":"Increased Circulating Levels of Interleukin-6 Induce Perturbation in Redox-Regulated Signaling Cascades in Muscle of Dystrophic Mice","volume":"2017","author":"Pelosi","year":"2017","journal-title":"Oxid. Med. Cell Longev."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"R345","DOI":"10.1152\/ajpregu.00454.2004","article-title":"Inflammatory processes in muscle injury and repair","volume":"288","author":"Tidball","year":"2005","journal-title":"Am. J. Physiol. Regul. Integr. Comp. Physiol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/0022-510X(84)90150-3","article-title":"Lipid peroxidation and superoxide dismutase activity in muscle and erythrocytes in Duchenne muscular dystrophy","volume":"63","author":"Mechler","year":"1984","journal-title":"J. Neurol. Sci."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1002\/mus.880100208","article-title":"Superoxide dismutases, glutathione peroxidase, and catalase in neuromuscular disease","volume":"10","author":"Burr","year":"1987","journal-title":"Muscle Nerve"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.2353\/ajpath.2010.090479","article-title":"Delayed cardiomyopathy in dystrophin deficient mdx mice relies on intrinsic glutathione resource","volume":"177","author":"Khouzami","year":"2010","journal-title":"Am. J. Pathol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1007\/s11064-011-0683-z","article-title":"Oxidative damage in muscular dystrophy correlates with the severity of the pathology: role of glutathione metabolism","volume":"37","author":"Renjini","year":"2012","journal-title":"Neurochem. Res."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s10974-012-9330-9","article-title":"Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy","volume":"34","author":"Kim","year":"2013","journal-title":"J. Muscle Res. Cell Motil."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.2353\/ajpath.2006.050683","article-title":"Sarcolemmal damage in dystrophin deficiency is modulated by synergistic interactions between mechanical and oxidative\/nitrosative stresses","volume":"168","author":"Dudley","year":"2006","journal-title":"Am. J. Pathol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1111\/j.1440-1681.2006.04394.x","article-title":"Muscle damage in mdx (dystrophic) mice: role of calcium and reactive oxygen species","volume":"33","author":"Whitehead","year":"2006","journal-title":"Clin. Exp. Pharmacol. Physiol."},{"key":"ref_68","doi-asserted-by":"crossref","unstructured":"Whitehead, N.P., Yeung, E.W., Froehner, S.C., and Allen, D.G. (2010). Skeletal muscle NADPH oxidase is increased and triggers stretch-induced damage in the mdx mouse. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0015354"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"e31732","DOI":"10.7554\/eLife.31732","article-title":"NADPH oxidase mediates microtubule alterations and diaphragm dysfunction in dystrophic mice","volume":"7","author":"Loehr","year":"2018","journal-title":"eLife"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"12864","DOI":"10.1073\/pnas.1509536112","article-title":"A new therapeutic effect of simvastatin revealed by functional improvement in muscular dystrophy","volume":"112","author":"Whitehead","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"991","DOI":"10.3109\/10715762.2011.590136","article-title":"Screening for increased protein thiol oxidation in oxidatively stressed muscle tissue","volume":"45","author":"Armstrong","year":"2011","journal-title":"Free Radic. Res."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1007\/978-0-387-73693-8_80","article-title":"Oxidative stress impairs upper airway muscle endurance in an animal model of sleep-disordered breathing","volume":"605","author":"Dunleavy","year":"2008","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_73","first-page":"425","article-title":"Role of N-acetylcysteine in the management of COPD","volume":"1","author":"Sadowska","year":"2006","journal-title":"Int. J. Chron. Obstruct. Pulmon. Dis."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1567","DOI":"10.1164\/ajrccm.156.5.96-09133","article-title":"Effect of N-acetylcysteine on human diaphragm strength and fatigability","volume":"156","author":"Travaline","year":"1997","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1113\/JP275598","article-title":"Antioxidant therapy for muscular dystrophy: caveat lector!","volume":"596","author":"Murphy","year":"2018","journal-title":"J. Physiol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1111\/j.1582-4934.2006.tb00297.x","article-title":"N-acetyl-L-cysteine abrogates fibrogenic properties of fibroblasts isolated from Dupuytren\u2019s disease by blunting TGF-beta signalling","volume":"10","author":"Kopp","year":"2006","journal-title":"J. Cell Mol. Med."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"e12757","DOI":"10.14814\/phy2.12757","article-title":"N-acetylcysteine attenuates the development of cardiac fibrosis and remodeling in a mouse model of heart failure","volume":"4","author":"Giam","year":"2016","journal-title":"Physiol. Rep."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1016\/j.jvs.2013.10.098","article-title":"Effects of N-acetylcysteine on skeletal muscle structure and function in a mouse model of peripheral arterial insufficiency","volume":"61","author":"Roseguini","year":"2015","journal-title":"J. Vasc. Surg."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"17718","DOI":"10.1038\/s41598-017-17927-5","article-title":"N-Acetylcysteine Attenuates the Development of Renal Fibrosis in Transgenic Mice with Dilated Cardiomyopathy","volume":"7","author":"Giam","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"4103","DOI":"10.1113\/JP278371","article-title":"Reply from David P. Burns, Eric F. Lucking and Ken D. O\u2019Halloran: Auxiliary compensation for diaphragm dysfunction in dystrophic disease","volume":"597","author":"Burns","year":"2019","journal-title":"J. Physiol."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"252","DOI":"10.3389\/fphys.2015.00252","article-title":"Pre- and postsynaptic changes in the neuromuscular junction in dystrophic mice","volume":"6","author":"Pratt","year":"2015","journal-title":"Front. Physiol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1623","DOI":"10.1111\/ejn.13249","article-title":"Characterization of neuromuscular synapse function abnormalities in multiple Duchenne muscular dystrophy mouse models","volume":"43","author":"Niks","year":"2016","journal-title":"Eur. J. Neurosci."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"7015","DOI":"10.1113\/JP275232","article-title":"Antioxidant therapy in a mouse model of Duchenne muscular dystrophy: some promising results but with a weighty caveat","volume":"595","author":"Head","year":"2017","journal-title":"J. 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