{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,11]],"date-time":"2026-11-11T05:03:51Z","timestamp":1794373431973,"version":"4.9.0"},"reference-count":38,"publisher":"National Academy of Sciences","issue":"42","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2010,10,19]]},"abstract":"<jats:p>\n                    Rett syndrome (RTT) is an autism spectrum disorder caused by mutations in the X-linked gene that encodes the transcription factor methyl-CpG-binding protein 2 (MeCP2). A major debilitating phenotype in affected females is frequent apneas, and heterozygous\n                    <jats:italic>Mecp2<\/jats:italic>\n                    -deficient female mice mimic the human respiratory disorder. GABA defects have been demonstrated in the brainstem of Mecp2-deficient mice. Here, using an intact respiratory network, we show that apnea in RTT mice is characterized by excessive excitatory activity in expiratory cranial and spinal nerves. Augmenting GABA markedly improves the respiratory phenotype. In addition, a serotonin 1a receptor agonist that depresses expiratory neuron activity also reduces apnea, corrects the irregular breathing pattern, and prolongs survival in MeCP2 null males. Combining a GABA reuptake blocker with a serotonin 1a agonist in heterozygous females completely corrects their respiratory defects. The results indicate that GABA and serotonin 1a receptor activity are candidates for treatment of the respiratory disorders in Rett syndrome.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1012104107","type":"journal-article","created":{"date-parts":[[2010,10,4]],"date-time":"2010-10-04T22:41:48Z","timestamp":1286232108000},"page":"18208-18213","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":165,"title":["Correction of respiratory disorders in a mouse model of Rett syndrome"],"prefix":"10.1073","volume":"107","author":[{"given":"Ana P. L.","family":"Abdala","sequence":"first","affiliation":[{"name":"Department of Physiology and Pharmacology, Bristol Heart Institute, School of Medical Sciences, University of Bristol, Bristol BS8 1TD, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathias","family":"Dutschmann","sequence":"additional","affiliation":[{"name":"Institute of Membrane and Systems Biology, Biological Sciences, University of Leeds, Leeds LS2 9JT, United Kingdom; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John M.","family":"Bissonnette","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Oregon Health and Sciences University, Portland, OR 97210"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian F. R.","family":"Paton","sequence":"additional","affiliation":[{"name":"Department of Physiology and Pharmacology, Bristol Heart Institute, School of Medical Sciences, University of Bristol, Bristol BS8 1TD, United Kingdom;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2010,10,4]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1042\/BST0360575"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2007.10.001"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2010.01.030"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1138389"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.63.9.1039"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1203\/01.pdr.0000238302.84552.d0"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.resp.2009.04.017"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2006.119966"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-0-387-73693-8_88"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00826.2007"},{"key":"e_1_3_3_12_2","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1113\/jphysiol.1994.sp020116","article-title":"5-HT-1A receptor-mediated modulation of medullary expiratory neurones in the cat","volume":"476","author":"Lalley PM","year":"1994","unstructured":"PM Lalley, AM Bischoff, DW Richter, 5-HT-1A receptor-mediated modulation of medullary expiratory neurones in the cat. 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