{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,4]],"date-time":"2026-04-04T18:02:10Z","timestamp":1775325730275,"version":"3.50.1"},"reference-count":55,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,1,13]],"date-time":"2019-01-13T00:00:00Z","timestamp":1547337600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001636","name":"University College Cork","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001636","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["physoc.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:sec>\n                    <jats:title>Key points<\/jats:title>\n                    <jats:p>\n                      <jats:list list-type=\"bullet\">\n                        <jats:list-item>\n                          <jats:p>Respiratory muscle weakness is a major feature of Duchenne muscular dystrophy (DMD), yet little is known about the neural control of the respiratory muscles in DMD and animal models of dystrophic disease.<\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>\n                            Substantial diaphragm muscle weakness is apparent in young (8\u2010week\u2010old)\n                            <jats:italic>mdx<\/jats:italic>\n                            mice, although ventilatory capacity in response to maximum chemostimulation in conscious mice is preserved.\n                          <\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>\n                            Peak volume\u2010 and flow\u2010related measures during chemoactivation are equivalent in anaesthetized, vagotomized wild\u2010type and\n                            <jats:italic>mdx<\/jats:italic>\n                            mice.\n                          <\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>\n                            Diaphragm and T3 external intercostal electromyogram activities are lower during protracted sustained airway occlusion in\n                            <jats:italic>mdx<\/jats:italic>\n                            compared to wild\u2010type mice. Yet, peak inspiratory pressure generation is remarkably well preserved.\n                          <\/jats:p>\n                        <\/jats:list-item>\n                        <jats:list-item>\n                          <jats:p>\n                            Despite profound diaphragm weakness and lower muscle activation during maximum non\u2010ventilatory efforts, inspiratory pressure\u2010generating capacity is preserved in young adult\n                            <jats:italic>mdx<\/jats:italic>\n                            mice, revealing compensation in support of respiratory system performance that is adequate, at least early in dystrophic disease.\n                          <\/jats:p>\n                        <\/jats:list-item>\n                      <\/jats:list>\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Abstract<\/jats:title>\n                    <jats:p>\n                      Diaphragm dysfunction is recognized in the\n                      <jats:italic>mdx<\/jats:italic>\n                      mouse model of muscular dystrophy; however, there is a paucity of information concerning the neural control of dystrophic respiratory muscles. In young adult (8\u00a0weeks of age) male wild\u2010type and\n                      <jats:italic>mdx<\/jats:italic>\n                      mice, we assessed ventilatory capacity, neural activation of the diaphragm and external intercostal (EIC) muscles and inspiratory pressure\u2010generating capacity during ventilatory and non\u2010ventilatory behaviours. We hypothesized that respiratory muscle weakness is associated with impaired peak inspiratory pressure\u2010generating capacity in\n                      <jats:italic>mdx<\/jats:italic>\n                      mice. Ventilatory responsiveness to hypercapnic hypoxia was determined in conscious mice by whole\u2010body plethysmography. Diaphragm isometric and isotonic contractile properties were determined\n                      <jats:italic>ex vivo<\/jats:italic>\n                      . In anaesthetized mice, thoracic oesophageal pressure, and diaphragm and EIC electromyogram (EMG) activities were recorded during baseline conditions and sustained tracheal occlusion for 30\u201340s. Despite substantial diaphragm weakness,\n                      <jats:italic>mdx<\/jats:italic>\n                      mice retain the capacity to enhance ventilation during hypercapnic hypoxia. Peak volume\u2010 and flow\u2010related measures were also maintained in anaesthetized, vagotomized\n                      <jats:italic>mdx<\/jats:italic>\n                      mice. Peak inspiratory pressure was remarkably well preserved during chemoactivated breathing, augmented breaths and maximal sustained efforts during airway obstruction in\n                      <jats:italic>mdx<\/jats:italic>\n                      mice. Diaphragm and EIC EMG activities were lower during airway obstruction in\n                      <jats:italic>mdx<\/jats:italic>\n                      compared to wild\u2010type mice. We conclude that ventilatory capacity is preserved in young\n                      <jats:italic>mdx<\/jats:italic>\n                      mice. Despite profound respiratory muscle weakness and lower diaphragm and EIC EMG activities during high demand in\n                      <jats:italic>mdx<\/jats:italic>\n                      mice, peak inspiratory pressure is preserved, revealing adequate compensation in support of respiratory system performance, at least early in dystrophic disease. We suggest that a progressive loss of compensation during advancing disease, combined with diaphragm dysfunction, underpins the development of respiratory system morbidity in dystrophic diseases.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1113\/jp277443","type":"journal-article","created":{"date-parts":[[2018,12,20]],"date-time":"2018-12-20T05:44:22Z","timestamp":1545284662000},"page":"831-848","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Inspiratory pressure\u2010generating capacity is preserved during ventilatory and non\u2010ventilatory behaviours in young dystrophic\n                    <i>mdx<\/i>\n                    mice despite profound diaphragm muscle weakness"],"prefix":"10.1113","volume":"597","author":[{"given":"David P.","family":"Burns","sequence":"first","affiliation":[{"name":"Department of Physiology School of Medicine College of Medicine and Health University 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