{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T04:43:00Z","timestamp":1792730580833,"version":"4.5.0"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,1,29]],"date-time":"2013-01-29T00:00:00Z","timestamp":1359417600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurogastroenterology Motil"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Hirschsprung's disease (<jats:styled-content style=\"fixed-case\">HSCR<\/jats:styled-content>) is a congenital condition in which enteric ganglia, formed from neural crest cells (<jats:styled-content style=\"fixed-case\">NCC<\/jats:styled-content>), are absent from the terminal bowel. Dysmotility and constipation are common features of <jats:styled-content style=\"fixed-case\">HSCR<\/jats:styled-content> that persist following surgical intervention. This persistence suggests that the portion of the colon that remains postoperatively is not able to support normal bowel function. To elucidate the defects that underlie this condition, we utilized a murine model of <jats:styled-content style=\"fixed-case\">HSCR<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Mice with <jats:styled-content style=\"fixed-case\">NCC<\/jats:styled-content>\u2010specific deletion of <jats:italic>Ednrb<\/jats:italic> were used to measure the neuronal density and neurotransmitter expression in ganglia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Key Results<\/jats:title><jats:p>At the site located proximal to the aganglionic region of P21 <jats:italic>Ednrb<\/jats:italic> null mice, the neuronal density is significantly decreased and the expression of neurotransmitters is altered compared with het animals. The ganglia in this colonic region are smaller and more isolated while the size of neuronal cell bodies is increased. The percentage of neurons expressing neuronal <jats:styled-content style=\"fixed-case\">nNOS<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">VIP<\/jats:styled-content> is significantly increased in <jats:italic>Ednrb<\/jats:italic> nulls. Conversely, the percentage of choline acetyltransferase (<jats:styled-content style=\"fixed-case\">ChAT<\/jats:styled-content>) expressing neurons is decreased, while Substance <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content> is unchanged between the two genotypes. These changes are limited to the colon and are not detected in the ileum.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions &amp; Inferences<\/jats:title><jats:p>We demonstrate changes in neuronal density and alterations in the balance of expression of neurotransmitters in the colon proximal to the aganglionic region in <jats:italic>Ednrb<\/jats:italic> null mice. The reduced neuronal density and complementary changes in <jats:styled-content style=\"fixed-case\">nNOS<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">ChAT<\/jats:styled-content> expression may account for the dysmotility seen in <jats:styled-content style=\"fixed-case\">HSCR<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nmo.12083","type":"journal-article","created":{"date-parts":[[2013,1,30]],"date-time":"2013-01-30T01:01:21Z","timestamp":1359507681000},"source":"Crossref","is-referenced-by-count":54,"title":["Altered neuronal density and neurotransmitter expression in the ganglionated region of <i>Ednrb<\/i> null mice: implications for Hirschsprung's disease"],"prefix":"10.1111","volume":"25","author":[{"given":"I.","family":"Zaitoun","sequence":"first","affiliation":[{"name":"Department of Neuroscience University of Wisconsin School of Medicine and Public Health Madison WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. 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F.","family":"Pierre","sequence":"additional","affiliation":[{"name":"Department of Surgery University of Wisconsin School of Medicine and Public Health Madison WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. L.","family":"Epstein","sequence":"additional","affiliation":[{"name":"Department of Neuroscience University of Wisconsin School of Medicine and Public Health Madison WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Gosain","sequence":"additional","affiliation":[{"name":"Department of Surgery University of Wisconsin School of Medicine and Public Health Madison WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,1,29]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2007.053959"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.sempedsurg.2010.03.004"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/00008480-200012000-00017"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0749.2001.140305.x"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn2137"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v17.i45.4937"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pneurobio.2012.01.001"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.jhered.a107457"},{"key":"e_1_2_9_10_1","first-page":"573","article-title":"Embryogenesis of the enteric ganglia in normal mice and in mice that develop congenital aganglionic megacolon","volume":"30","author":"Webster W","year":"1973","journal-title":"J Embryol Exp Morphol"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90018-3"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90017-5"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.00420.2005"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1015085923245"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.00558.2007"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/dvg.20415"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.031302"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1679\/aohc.53.467"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ydbio.2006.03.031"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.22755"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/0012-1606(88)90217-5"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.51.3.414"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.902570209"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3861-09.2010"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004410050565"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00441-008-0684-7"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2982.2011.01780.x"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.21770"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0185(199806)251:2<185::AID-AR6>3.0.CO;2-Y"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1582-4934.2009.00813.x"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.903140411"}],"container-title":["Neurogastroenterology &amp; Motility"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fnmo.12083","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nmo.12083","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T19:30:57Z","timestamp":1698694257000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/nmo.12083"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,1,29]]},"references-count":30,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["10.1111\/nmo.12083"],"URL":"https:\/\/doi.org\/10.1111\/nmo.12083","archive":["Portico"],"relation":{},"ISSN":["1350-1925","1365-2982"],"issn-type":[{"value":"1350-1925","type":"print"},{"value":"1365-2982","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,1,29]]}}}