scholarly journals Mice Lacking Zfhx1b, the Gene That Codes for Smad-Interacting Protein-1, Reveal a Role for Multiple Neural Crest Cell Defects in the Etiology of Hirschsprung Disease–Mental Retardation Syndrome

2003 ◽  
Vol 72 (2) ◽  
pp. 465-470 ◽  
Author(s):  
Tom Van de Putte ◽  
Mitsuji Maruhashi ◽  
Annick Francis ◽  
Luc Nelles ◽  
Hisato Kondoh ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Neural Crest ◽  
Neural Crest Cell ◽  
Hirschsprung Disease ◽  
Interacting Protein ◽  
Crest Cell

scholarly journals Skip segment Hirschsprung disease: modelling the trans-mesenteric origin of the enteric nervous system in the human colon

2019 ◽  
Author(s):  
Donald F Newgreen ◽  
James M Osborne ◽  
Dongcheng Zhang
Keyword(s):  
Cell Migration ◽  
Neural Crest ◽  
Growth Parameters ◽  
Neural Crest Cell ◽  
Hirschsprung Disease ◽  
Division Rate ◽  
Intestinal Growth ◽  
Developmental Anatomy ◽  
Neural Crest Cell Migration ◽  
Crest Cell

ABSTRACTSkip segment Hirschsprung disease is a difficult to explain human enteric neuropathy where a ganglionated region lies within a region of total colonic aganglionosis.. Recently, trans-mesenteric migration was described in the mouse intestine whereby neural crest cells migrate via the mesentery across a U-shape gut loop from the midgut to the hindgut: this could explain skip segment Hirschsprung disease. To investigate this, human intestinal growth parameters were derived from published sources and correlated with enteric neural crest cell migration. These processes were then simulated using agent based mathematical models scaled to human intestinal growth. A Hirschsprung-associated slowing of migration was imposed and trans-mesenteric migration was allowed. From the developmental anatomy we conclude that trans-mesenteric migration is unlikely in normal human embryogenesis, but with a Hirschsprung-associated slowing of enteric neural crest cell migration it could occur at Carnegie stages 17 and 18. By varying the division rate of enteric neural crest agents we could reproduce full colonisation, short segment, long segment and skip segment Hirschsprung and hypoganglionic segments.Summary StatementSkip segment Hirschsprung disease in humans challenges current explanations. Mathematical modelling shows how this birth defect could develop.

Early Death of Neural Crest Cells is Responsible for Total Enteric Aganglionosis in Sox10Dom/Sox10Dom Mouse Embryos

1999 ◽  
Vol 2 (6) ◽  
pp. 559-569 ◽  
Author(s):  
Raj P. Kapur
Keyword(s):  
Neural Crest ◽  
Neural Crest Cell ◽  
Hirschsprung Disease ◽  
Early Death ◽  
Neural Crest Cells ◽  
Wild Type ◽  
Crest Cell ◽  
Insight Into ◽  
Intestinal Aganglionosis ◽  
Migratory Pathway

Intestinal aganglionosis results from homologous genetic defects in humans and mice, including mutations of Sox10, which encodes a transcription factor expressed in neural crest cells. To gain insight into the embryological basis for this condition, the phenotype and pathogenesis of intestinal aganglionosis in Sox10 Dom/ Sox10 Dom embryos were studied. The distribution of enteric neural precursors and other neural crest derivatives in Sox10 Dom/ Sox10 Dom embryos was analyzed with immunochemical and transgenic markers. The ability of wild-type neural crest cells to colonize Sox10 Dom/ Sox10 Dom intestinal ex-plants was evaluated by appositional grafts under the renal capsule. Apoptosis was studied by TUNEL labeling. Sox10 Dom/ Sox10 Dom embryos died pre- or perinatally with total enteric aganglionosis and hypoplasia or agenesis of nonenteric ganglia. Mutant crest cells failed to colonize any portion of the Sox10 Dom/ Sox10 Dom gut, but wild-type neural crest cells were able to colonize explanted segments of Sox10 Dom/ Sox10 Dom embryonic intestine. In Sox10 Dom/ Sox10 Dom embryos, apoptosis was increased in sites of early neural crest cell development, before these cells enter the gut. Sox10 Dom/ Sox10 Dom embryos are one of many genetic animal models for human Hirschsprung disease. The underlying problem is probably not the enteric microenvironment, since Sox10 Dom/ Sox10 Dom intestine supports colonization and neuronal differentiation by wild-type neural crest cells. Instead, excessive cell death occurs in mutant neural crest cells early in their migratory pathway. Comparison with other models suggests that genetic heterogeneity of aganglionosis correlates with different pathogenetic mechanisms.

scholarly journals Proliferation dynamics associated with cranial neural crest cell migration

2011 ◽  
Vol 356 (1) ◽  
pp. 197
Author(s):  
Dennis A. Ridenour ◽  
Rebecca McLennan ◽  
Jessica M. Teddy ◽  
Katherine W. Prather ◽  
Craig L. Semerad ◽  
...  
Keyword(s):  
Cell Migration ◽  
Neural Crest ◽  
Neural Crest Cell ◽  
Cranial Neural Crest ◽  
Cranial Neural Crest Cell ◽  
Neural Crest Cell Migration ◽  
Crest Cell

Analysis of developmentally homogeneous neural crest cell populations in vitro

1981 ◽  
Vol 82 (1) ◽  
pp. 86-94 ◽  
Author(s):  
Jeanne Loring ◽  
Bengt Glimelius ◽  
Carol Erickson ◽  
James A. Weston
Keyword(s):  
Neural Crest ◽  
Neural Crest Cell ◽  
Cell Populations ◽  
Crest Cell
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