{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T05:55:02Z","timestamp":1781848502090,"version":"3.54.5"},"reference-count":81,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T00:00:00Z","timestamp":1781827200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cell. Neurosci."],"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>\n                      Spinal muscular atrophy (SMA) is a neurodegenerative disorder caused by\n                      <jats:italic>SMN1<\/jats:italic>\n                      mutations, leading to SMN protein deficiency and motor neuron loss. While progressive weakness, respiratory defects, and oral dysfunction are well-documented in patients, the underlying pathophysiology of breathing and bulbar deficits remains understudied in SMA animal models.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We evaluated breathing and oral function in the SMN\u22067 mouse model of severe SMA. Respiratory parameters and chemoreflexes were assessed via whole-body plethysmography. To identify underlying structural changes, we performed histological analysis on lung tissue, the phrenic and hypoglossal nerves, and the muscles driving respiration and oral function.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>SMN\u22067 mice exhibited baseline respiratory alterations and chemoreflex deficits. Histological analysis revealed reduced neuromuscular junction (NMJ) occupancy in respiratory and oral muscles, alongside axonal pathology in the phrenic and hypoglossal nerves and structural degradation in lung tissue.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>These data provide the first physiological and histological evidence of linked respiratory and oral dysfunction in the SMN\u22067 mouse. Because these deficits closely approximate the clinical presentation seen in SMA patients, this model represents a valuable tool for testing therapies targeted at bulbar and respiratory failure.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fncel.2026.1844362","type":"journal-article","created":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T05:48:42Z","timestamp":1781848122000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["SM\u039d\u03947 mice show breathing and airflow defects with significant pathology of respiratory and oral tract tissues"],"prefix":"10.3389","volume":"20","author":[{"given":"Roxanne","family":"Muchow","sequence":"first","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Woolridge","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine L.","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F. Javier","family":"Llorente Torres","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis","family":"Perez-Lopez","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole L.","family":"Nichols","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monique A.","family":"Lorson","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian L.","family":"Lorson","sequence":"additional","affiliation":[{"name":"Department of Pathobiology and Integrative Biomedical Sciences, College of Veterinary Medicine, University of Missouri","place":["Columbia, MO, United States"]},{"name":"Bond Life Sciences Center, University of Missouri","place":["Columbia, MO, United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,19]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1299205","DOI":"10.3389\/fneur.2024.1299205","article-title":"Sleep and sleep-related breathing disorders in patients with spinal muscular atrophy: a changing perspective from novel treatments?","volume":"15","author":"Abati","year":"2024","journal-title":"Front. 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