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Accurate descriptions of the phenotype of ENS dysplasias, and knowledge of genes which, when mutated, give rise to the disorders (see Part 1 in the previous issue of this journal), are not sufficient to give a real understanding of how these abnormalities arise. The often indirect link between genotype and phenotype must be sought in the early embryonic development of the ENS. Therefore, in this, the second part, we provide a description of the development of the ENS, concentrating mainly on the origin of the ENS precursor cells and on the cell migration by which they become distributed throughout the gastrointestinal tract. This section also includes experimental evidence on the controls of ENS formation derived from classic embryological, cell culture, and molecular genetic approaches. In addition, for reasons of completeness, we also briefly describe the origins of the interstitial cells of Cajal, a cell population closely related anatomically and functionally to the ENS. Finally, a brief sketch is presented of current notions on the developmental processes between the genes and the morphogenesis of the ENS, and of the means by which the known genetic abnormalities might result in the ENS phenotype observed in Hirschsprung's disease. <\/jats:p>","DOI":"10.1007\/s10024-002-0002-4","type":"journal-article","created":{"date-parts":[[2002,10,14]],"date-time":"2002-10-14T16:45:16Z","timestamp":1034613916000},"page":"329-349","source":"Crossref","is-referenced-by-count":155,"title":["Enteric Nervous System: Development and Developmental Disturbances\u2014Part 2"],"prefix":"10.1177","volume":"5","author":[{"given":"Donald","family":"Newgreen","sequence":"first","affiliation":[{"name":"Murdoch Children's Research Institute, Royal Children's Hospital, Parkville, 3052, Victoria, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heather M.","family":"Young","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell 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