{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T03:45:06Z","timestamp":1790739906517,"version":"4.1.0"},"reference-count":81,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,12,15]],"date-time":"2020-12-15T00:00:00Z","timestamp":1607990400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82070527"],"award-info":[{"award-number":["82070527"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004731","name":"Natural Science Foundation of Zhejiang Province","doi-asserted-by":"publisher","award":["LY19C090005"],"award-info":[{"award-number":["LY19C090005"]}],"id":[{"id":"10.13039\/501100004731","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2017YFA0104200"],"award-info":[{"award-number":["2017YFA0104200"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012226","name":"Fundamental Research Funds for the Central Universities","doi-asserted-by":"publisher","award":["2019FZA7009"],"award-info":[{"award-number":["2019FZA7009"]}],"id":[{"id":"10.13039\/501100012226","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","cmghjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cellular and Molecular Gastroenterology and Hepatology"],"published-print":{"date-parts":[[2021]]},"DOI":"10.1016\/j.jcmgh.2020.12.010","type":"journal-article","created":{"date-parts":[[2020,12,17]],"date-time":"2020-12-17T02:36:48Z","timestamp":1608172608000},"page":"1-24","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":21,"title":["Mesenteric Neural Crest Cells Are the Embryological Basis of Skip Segment Hirschsprung\u2019s Disease"],"prefix":"10.1016","volume":"12","author":[{"given":"Qi","family":"Yu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengjie","family":"Du","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhigang","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Gu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kun","family":"Zhu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xueyuan","family":"Niu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiming","family":"Sun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9343-9730","authenticated-orcid":false,"given":"Liang","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jcmgh.2020.12.010_bib1","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/cne.901010212","article-title":"The origin of intrinsic ganglia of trunk viscera from vagal neural crest in the chick embryo","volume":"101","author":"Yntema","year":"1954","journal-title":"J\u00a0Comp Neurol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib2","first-page":"31","article-title":"The migration of neural crest cells to the wall of the digestive tract in avian embryo","volume":"30","author":"Le Douarin","year":"1973","journal-title":"J\u00a0Embryol Exp Morphol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib3","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1038\/nrgastro.2012.234","article-title":"Development and developmental disorders of the enteric nervous system","volume":"10","author":"Obermayr","year":"2013","journal-title":"Nat Rev Gastroenterol Hepatol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib4","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1038\/nrgastro.2017.149","article-title":"Hirschsprung disease - integrating basic science and clinical medicine to improve outcomes","volume":"15","author":"Heuckeroth","year":"2018","journal-title":"Nat Rev Gastroenterol Hepatol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib5","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1007\/s00383-010-2692-4","article-title":"Skip segment Hirschsprung's disease: a systematic review","volume":"26","author":"O'Donnell","year":"2010","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib6","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1007\/s00383-015-3742-8","article-title":"Rectal suction biopsy for the diagnosis of Hirschsprung's disease: a systematic review of diagnostic accuracy and complications","volume":"31","author":"Friedmacher","year":"2015","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib7","doi-asserted-by":"crossref","first-page":"123","DOI":"10.2350\/15-08-1686-OA.1","article-title":"Distal rectal skip-segment Hirschsprung disease and the potential for false-negative diagnosis","volume":"19","author":"Coe","year":"2016","journal-title":"Pediatr Dev Pathol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib8","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1148\/63.2.157","article-title":"Congenital megacolon (Hirschsprung's disease)","volume":"63","author":"Keefer","year":"1954","journal-title":"Radiology"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib9","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1097\/00000658-196101000-00017","article-title":"Hirschsprung's disease with skip area","volume":"153","author":"Sprinz","year":"1961","journal-title":"Ann Surg"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib10","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1136\/adc.47.252.233","article-title":"Zonal colonic aganglionosis, a variant of Hirschsprung's disease","volume":"47","author":"MacIver","year":"1972","journal-title":"Arch Dis Child"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib11","doi-asserted-by":"crossref","first-page":"686","DOI":"10.1016\/S0022-3468(79)80245-6","article-title":"Hirschsprung\u2019s disease with skip area (segmental aganglionosis)","volume":"14","author":"Martin","year":"1979","journal-title":"J Pediatr Surg"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib12","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/S0022-3468(82)80213-3","article-title":"Double zonal aganglionosis in long segment Hirschsprung's disease with a \u201cskip area\u201d in transverse colon","volume":"17","author":"de Chadarevian","year":"1982","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib13","doi-asserted-by":"crossref","first-page":"33","DOI":"10.3109\/15513818309048283","article-title":"Does zonal aganglionosis really exist? Report of a rare variety of Hirschsprung's disease and review of the literature","volume":"1","author":"Yunis","year":"1983","journal-title":"Pediatr Pathol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib14","first-page":"312","article-title":"Double zonal aganglionosis with a skipped oligoganglionic ascending colon","volume":"38","author":"Taguchi","year":"1983","journal-title":"Z\u00a0Kinderchir"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib15","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/BF00710909","article-title":"Zonal aganglionosis. An enzyme and immunohistochemical study of two cases","volume":"410","author":"Seldenrijk","year":"1986","journal-title":"Virchows Arch A Pathol Anat Histopathol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib16","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1016\/S0022-3468(86)80006-9","article-title":"Segmental aganglionosis of the appendix","volume":"21","author":"Anderson","year":"1986","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib17","doi-asserted-by":"crossref","first-page":"23","DOI":"10.3109\/15513819509026937","article-title":"Hypothesis: pathogenesis of skip areas in long-segment Hirschsprung's disease","volume":"15","author":"Kapur","year":"1995","journal-title":"Pediatr Pathol Lab Med"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib18","first-page":"2772","article-title":"[Clinical study of multiple zonal aganglionosis in long segment Hirschsprung's disease]","volume":"85","author":"Yang","year":"2005","journal-title":"Zhonghua Yi Xue Za Zhi"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib19","doi-asserted-by":"crossref","first-page":"200","DOI":"10.5144\/0256-4947.2006.200","article-title":"Clinicopathological features in 102 cases of Hirschsprung disease","volume":"26","author":"Ziad","year":"2006","journal-title":"Ann Saudi Med"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib20","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1016\/j.jpedsurg.2007.09.005","article-title":"Imperforate anus, malrotation, and Hirschsprung's disease with double zonal aganglionosis: an extremely rare combination","volume":"43","author":"Oshio","year":"2008","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib21","unstructured":"O'Donnell AM, Puri P. Skip segment Hirschsprung\u2019s disease: a rare phenomenon. European Union Paediatric Surgery Association 2010 Meeting 2010; Berne, Switzerland."},{"key":"10.1016\/j.jcmgh.2020.12.010_bib22","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1007\/s00383-011-2903-7","article-title":"Skip segment Hirschsprung's disease: a rare phenomenon","volume":"27","author":"Doi","year":"2011","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib23","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.ijscr.2012.02.004","article-title":"'Hop the skip' with extended segment intestinal biopsy in Hirschsprung's disease","volume":"3","author":"Burjonrappa","year":"2012","journal-title":"Int J Surg Case Rep"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib24","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1007\/s00383-013-3286-8","article-title":"Segmental aganglionosis (zonal aganglionosis or \"skip\" lesions) in Hirschsprungs disease: a report of 2 unusual cases","volume":"29","author":"Moore","year":"2013","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib25","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00383-013-3428-z","article-title":"A\u00a0rare case of multiple skip segment Hirschsprung's disease in the ileum and colon","volume":"30","author":"Erten","year":"2014","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib26","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1007\/s00383-013-3367-8","article-title":"Skip segment Hirschsprung's disease: a case report and novel management technique","volume":"30","author":"Raghunath","year":"2014","journal-title":"Pediatr Surg Int"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib27","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.epsc.2015.02.005","article-title":"Skip segment Hirschsprung disease and Waardenburg syndrome","volume":"3","author":"Gross","year":"2015","journal-title":"J\u00a0Pediatr Surg Case Rep"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib28","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.epsc.2016.08.012","article-title":"Skip segment Hirschsprung's disease in a patient with Shah-Waardenburg Syndrome","volume":"15","author":"Ruiz","year":"2016","journal-title":"J\u00a0Pediatr Surg Case Rep"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib29","first-page":"25","article-title":"Skip segment Hirschsprungs disease: avoiding the potential pitfall of a failed pull-through procedure","volume":"4","author":"Alfawaz","year":"2017","journal-title":"EC Paediatrics Special Issue"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib30","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1080\/15513815.2019.1608606","article-title":"Distal rectal skip segment Hirschsprung disease: case report and review of literature","volume":"38","author":"Shenoy","year":"2019","journal-title":"Fetal Pediatr Pathol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib31","doi-asserted-by":"crossref","first-page":"4335","DOI":"10.1242\/dev.125.21.4335","article-title":"The sacral neural crest contributes neurons and glia to the post-umbilical gut: spatiotemporal analysis of the development of the enteric nervous system","volume":"125","author":"Burns","year":"1998","journal-title":"Development"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib32","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1053\/j.gastro.2011.06.002","article-title":"Analysis of the sacral neural crest cell contribution to the hindgut enteric nervous system in the mouse\u00a0embryo","volume":"141","author":"Wang","year":"2011","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib33","first-page":"82","article-title":"Migration of ganglion cell precursors in the ileoceca of normal and lethal spotted embryos, a murine model for Hirschsprung disease","volume":"71","author":"Coventry","year":"1994","journal-title":"Lab Invest"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib34","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/j.ydbio.2005.08.040","article-title":"The pattern of neural crest advance in the cecum and colon","volume":"287","author":"Druckenbrod","year":"2005","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib35","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1038\/nn.3184","article-title":"Trans-mesenteric neural crest cells are the principal source of the colonic enteric nervous system","volume":"15","author":"Nishiyama","year":"2012","journal-title":"Nat Neurosci"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib36","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1126\/science.1230717","article-title":"Tissue interactions in neural crest cell development and disease","volume":"341","author":"Takahashi","year":"2013","journal-title":"Science"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib37","doi-asserted-by":"crossref","first-page":"9879","DOI":"10.1523\/JNEUROSCI.1239-15.2015","article-title":"Neuronal differentiation in Schwann cell lineage underlies postnatal neurogenesis in the enteric nervous system","volume":"35","author":"Uesaka","year":"2015","journal-title":"J\u00a0Neurosci"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib38","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1038\/nature21679","article-title":"Ancient evolutionary origin of vertebrate enteric neurons from trunk-derived neural crest","volume":"544","author":"Green","year":"2017","journal-title":"Nature"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib39","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1038\/nrn1746","article-title":"The origin and development of glial cells in peripheral nerves","volume":"6","author":"Jessen","year":"2005","journal-title":"Nat Rev Neurosci"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib40","doi-asserted-by":"crossref","first-page":"1471","DOI":"10.1016\/0022-3468(95)90410-7","article-title":"Spatial distribution and pattern of extrinsic nerve strands in the aganglionic segment of congenital aganglionosis: stereoscopic analysis in spotting lethal rats","volume":"30","author":"Watanabe","year":"1995","journal-title":"J\u00a0Pediatr Surg"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib41","doi-asserted-by":"crossref","first-page":"1826","DOI":"10.1073\/pnas.0337540100","article-title":"Phenotype variation in two-locus mouse models of Hirschsprung disease: tissue-specific interaction between Ret and Ednrb","volume":"100","author":"McCallion","year":"2003","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib42","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1016\/S2468-1253(16)30026-7","article-title":"The mesentery: structure, function, and role in disease","volume":"1","author":"Coffey","year":"2016","journal-title":"Lancet Gastroenterol Hepatol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib43","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/S0012-1606(03)00160-X","article-title":"The endothelin receptor-B is required for the migration of neural crest-derived melanocyte and enteric neuron precursors","volume":"259","author":"Lee","year":"2003","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib44","first-page":"4385","article-title":"Expanding the power of recombinase-based labeling to uncover cellular diversity","volume":"142","author":"Plummer","year":"2015","journal-title":"Development"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib45","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1002\/dvg.22393","article-title":"A\u00a0Phox2b::FLPo transgenic mouse line suitable for intersectional genetics","volume":"51","author":"Hirsch","year":"2013","journal-title":"Genesis"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib46","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/1097-0185(20010101)262:1<1::AID-AR1006>3.0.CO;2-2","article-title":"Enteric neural crest-derived cells: origin, identification, migration, and differentiation","volume":"262","author":"Young","year":"2001","journal-title":"Anat Rec"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib47","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1006\/dbio.1998.8987","article-title":"A\u00a0single rostrocaudal colonization of the rodent intestine by enteric neuron precursors is revealed by the expression of Phox2b, Ret, and p75 and by explants grown under the kidney capsule or in organ culture","volume":"202","author":"Young","year":"1998","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib48","doi-asserted-by":"crossref","first-page":"11980","DOI":"10.1073\/pnas.1710308114","article-title":"Dual origin of enteric neurons in vagal Schwann cell precursors and the sympathetic neural crest","volume":"114","author":"Espinosa-Medina","year":"2017","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib49","doi-asserted-by":"crossref","first-page":"6691","DOI":"10.1523\/JNEUROSCI.0309-20.2020","article-title":"Mapping of extrinsic innervation of the gastrointestinal tract in the mouse embryo","volume":"40","author":"Niu","year":"2020","journal-title":"J\u00a0Neurosci"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib50","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1016\/0092-8674(94)90017-5","article-title":"Targeted and natural (piebald-lethal) mutations of endothelin-B receptor gene produce megacolon associated with spotted coat color in mice","volume":"79","author":"Hosoda","year":"1994","journal-title":"Cell"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib51","doi-asserted-by":"crossref","first-page":"6005","DOI":"10.1523\/JNEUROSCI.22-14-06005.2002","article-title":"The projections of early enteric neurons are influenced by the direction of neural crest cell migration","volume":"22","author":"Young","year":"2002","journal-title":"J\u00a0Neurosci"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib52","doi-asserted-by":"crossref","DOI":"10.1002\/dvg.23105","article-title":"Cre-driver lines used for genetic fate mapping of neural crest cells in the mouse: an overview","volume":"56","author":"Debbache","year":"2018","journal-title":"Genesis"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib53","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1038\/nature13536","article-title":"Glial origin of mesenchymal stem cells in a tooth model system","volume":"513","author":"Kaukua","year":"2014","journal-title":"Nature"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib54","doi-asserted-by":"crossref","first-page":"3358","DOI":"10.1002\/cne.23354","article-title":"Development of myenteric cholinergic neurons in ChAT-Cre;R26R-YFP mice","volume":"521","author":"Hao","year":"2013","journal-title":"J\u00a0Comp Neurol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib55","first-page":"54161","article-title":"Improved Swiss-rolling technique for intestinal tissue preparation for immunohistochemical and immunofluorescent analyses","volume":"113","author":"Bialkowska","year":"2016","journal-title":"J\u00a0Vis Exp"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib56","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/S0002-9440(10)62250-X","volume":"166","author":"Skinner","year":"2005","journal-title":"Am J Pathol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib57","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.1016\/0092-8674(94)90018-3","article-title":"Interaction of endothelin-3 with endothelin-B receptor is essential for development of epidermal melanocytes and enteric neurons","volume":"79","author":"Baynash","year":"1994","journal-title":"Cell"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib58","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1038\/40146","article-title":"Wnt signalling required for expansion of neural crest and CNS progenitors","volume":"389","author":"Ikeya","year":"1997","journal-title":"Nature"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib59","doi-asserted-by":"crossref","first-page":"10050","DOI":"10.1073\/pnas.97.18.10050","article-title":"Neural crest-directed gene transfer demonstrates Wnt1 role in melanocyte expansion and differentiation during mouse development","volume":"97","author":"Dunn","year":"2000","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib60","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/j.ydbio.2013.04.026","article-title":"The widely used Wnt1-Cre transgene causes developmental phenotypes by ectopic activation of Wnt signaling","volume":"379","author":"Lewis","year":"2013","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib61","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1038\/367378a0","article-title":"Mutations of the RET proto-oncogene in Hirschsprung's disease","volume":"367","author":"Edery","year":"1994","journal-title":"Nature"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib62","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1038\/367380a0","article-title":"Defects in the kidney and enteric nervous system of mice lacking the tyrosine kinase receptor Ret","volume":"367","author":"Schuchardt","year":"1994","journal-title":"Nature"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib63","doi-asserted-by":"crossref","first-page":"2075","DOI":"10.1242\/dev.02375","article-title":"Maintenance of mammalian enteric nervous system progenitors by SOX10 and endothelin 3 signalling","volume":"133","author":"Bondurand","year":"2006","journal-title":"Development"},{"issue":"Suppl 1","key":"10.1016\/j.jcmgh.2020.12.010_bib64","doi-asserted-by":"crossref","first-page":"S156","DOI":"10.1016\/j.ydbio.2018.08.014","article-title":"News from the endothelin-3\/EDNRB signaling pathway: role during enteric nervous system development and involvement in neural crest-associated disorders","volume":"444","author":"Bondurand","year":"2018","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib65","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1006\/dbio.2000.0100","article-title":"GDNF is a chemoattractant for enteric neural cells","volume":"229","author":"Young","year":"2001","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib66","doi-asserted-by":"crossref","first-page":"5151","DOI":"10.1242\/dev.129.22.5151","article-title":"Requirement of signalling by receptor tyrosine kinase RET for the directed migration of enteric nervous system progenitor cells during mammalian embryogenesis","volume":"129","author":"Natarajan","year":"2002","journal-title":"Development"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib67","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1136\/gut.44.2.246","article-title":"Expression of endothelin 3 by mesenchymal cells of embryonic mouse caecum","volume":"44","author":"Leibl","year":"1999","journal-title":"Gut"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib68","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1038\/990040","article-title":"The temporal requirement for endothelin receptor-B signalling during neural crest development","volume":"402","author":"Shin","year":"1999","journal-title":"Nature"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib69","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1006\/dbio.1999.9592","article-title":"Sacral neural crest cells colonise aganglionic hindgut in\u00a0vivo but fail to compensate for lack of enteric ganglia","volume":"219","author":"Burns","year":"2000","journal-title":"Dev Biol"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib70","article-title":"Skip segment Hirschsprung disease: modelling the trans-mesenteric origin of the enteric nervous system in the human colon","volume":"1\u201324","author":"Newgreen","year":"2019","journal-title":"bioRxiv"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib71","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1136\/gut.39.5.765","article-title":"Zonal adult Hirschsprung's disease","volume":"39","author":"Fu","year":"1996","journal-title":"Gut"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib72","doi-asserted-by":"crossref","first-page":"2171","DOI":"10.1242\/dev.001388","article-title":"Conditional ablation of GFR alpha 1 in postmigratory enteric neurons triggers unconventional neuronal death in the colon and causes a Hirschsprung's disease phenotype","volume":"134","author":"Uesaka","year":"2007","journal-title":"Development"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib73","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1002\/(SICI)1098-1004(200005)15:5<418::AID-HUMU3>3.0.CO;2-2","article-title":"RET and GDNF gene scanning in Hirschsprung patients using two dual denaturing gel systems","volume":"15","author":"Hofstra","year":"2000","journal-title":"Hum Mutat"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib74","first-page":"303","article-title":"Endothelin receptor-mediated signaling in Hirschsprung disease","volume":"5","author":"Chakravarti","year":"1996","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib75","doi-asserted-by":"crossref","first-page":"1908","DOI":"10.1053\/j.gastro.2018.09.012","article-title":"Identification of genes associated with Hirschsprung disease, based on whole-genome sequence analysis, and potential effects on enteric nervous system development","volume":"155","author":"Tang","year":"2018","journal-title":"Gastroenterology"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib76","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1242\/dev.02838","article-title":"Specific ablation of the transcription factor CREB in sympathetic neurons surprisingly protects against developmentally regulated apoptosis","volume":"134","author":"Parlato","year":"2007","journal-title":"Development"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib77","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.neuron.2011.06.021","article-title":"Anatomical coupling of sensory and motor nerve trajectory via axon tracking","volume":"71","author":"Wang","year":"2011","journal-title":"Neuron"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib78","doi-asserted-by":"crossref","first-page":"16704","DOI":"10.1073\/pnas.0904336106","article-title":"The tyrosine phosphatase Shp2 (PTPN11) directs neuregulin-1\/ErbB signaling throughout Schwann cell development","volume":"106","author":"Grossmann","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib79","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1101\/gad.230045.113","article-title":"Activation of MAPK overrides the termination of myelin growth and replaces Nrg1\/ErbB3 signals during Schwann cell development and myelination","volume":"28","author":"Sheean","year":"2014","journal-title":"Gene Dev"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib80","doi-asserted-by":"crossref","first-page":"4065","DOI":"10.1242\/dev.124.20.4065","article-title":"Expression and interactions of the two closely related homeobox genes Phox2a and Phox2b during neurogenesis","volume":"124","author":"Pattyn","year":"1997","journal-title":"Development"},{"key":"10.1016\/j.jcmgh.2020.12.010_bib81","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.jmoldx.2011.08.002","article-title":"RNAscope a novel in situ RNA analysis platform for formalin-fixed, paraffin-embedded tissues","volume":"14","author":"Wang","year":"2012","journal-title":"J\u00a0Mol Diagn"}],"container-title":["Cellular and Molecular Gastroenterology and Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2352345X20302095?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2352345X20302095?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,6]],"date-time":"2025-11-06T18:27:58Z","timestamp":1762453678000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2352345X20302095"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021]]},"references-count":81,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021]]}},"alternative-id":["S2352345X20302095"],"URL":"https:\/\/doi.org\/10.1016\/j.jcmgh.2020.12.010","relation":{},"ISSN":["2352-345X"],"issn-type":[{"value":"2352-345X","type":"print"}],"subject":[],"published":{"date-parts":[[2021]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Mesenteric Neural Crest Cells Are the Embryological Basis of Skip Segment Hirschsprung\u2019s Disease","name":"articletitle","label":"Article Title"},{"value":"Cellular and Molecular Gastroenterology and Hepatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcmgh.2020.12.010","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 The Authors. Published by Elsevier Inc. on behalf of the AGA Institute.","name":"copyright","label":"Copyright"}]}}