{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T14:14:00Z","timestamp":1791036840788,"version":"4.1.0"},"reference-count":53,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,3,14]],"date-time":"2007-03-14T00:00:00Z","timestamp":1173830400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Physiology"],"published-print":{"date-parts":[[2007,3,15]]},"abstract":"<jats:p>Rett syndrome (RTT) is an inborn neurodevelopmental disorder caused by mutations in the X\u2010linked methyl\u2010CpG binding protein 2 gene (<jats:italic>MECP2<\/jats:italic>). Besides mental retardation, most patients suffer from potentially life\u2010threatening breathing arrhythmia. To study its pathophysiology, we performed comparative analyses of the breathing phenotype of <jats:italic>Mecp2<jats:sup>\u2212\/y<\/jats:sup><\/jats:italic> knockout (KO) and C57BL\/6J wild\u2010type mice using the perfused working heart\u2013brainstem preparation (WHBP). We simultaneously recorded phrenic and efferent vagal nerve activities to analyse the motor pattern of respiration, discriminating between inspiration, postinspiration and late expiration. Our results revealed respiratory disturbances in KO preparations that were similar to those reported from <jats:italic>in vivo<\/jats:italic> measurements in KO mice and also to those seen in RTT patients. The main finding was a highly variable postinspiratory activity in KO mice that correlated closely with breathing arrhythmias leading to repetitive apnoeas even under undisturbed control conditions. Analysis of the pontine and peripheral sensory regulation of postinspiratory activity in KO preparations revealed: (i) prolonged apnoeas associated with enhanced postinspiratory activity after glutamate\u2010induced activation of the pontine K\u00f6lliker\u2010Fuse nucleus; and (ii) prolonged apnoeas and lack of reflex desensitization in response to repetitive vagal stimulations. We conclude that impaired network and sensory mediated synaptic control of postinspiration induces severe breathing dysfunctions in <jats:italic>Mecp2<jats:sup>\u2212\/y<\/jats:sup><\/jats:italic> KO preparations. As postinspiration is particularly important for the control of laryngeal adductors, the finding might explain the upper airway\u2010related clinical problems of patients with RTT such as apnoeas, loss of speech and weak coordination of breathing and swallowing.<\/jats:p>","DOI":"10.1113\/jphysiol.2006.119966","type":"journal-article","created":{"date-parts":[[2007,1,5]],"date-time":"2007-01-05T01:53:12Z","timestamp":1167961992000},"page":"863-876","source":"Crossref","is-referenced-by-count":144,"title":["Breathing dysfunctions associated with impaired control of postinspiratory activity in <i>Mecp2<sup>\u2212\/y<\/sup><\/i> knockout mice"],"prefix":"10.1113","volume":"579","author":[{"given":"Georg 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