... region becomes non-functional and results in a condition in humans known as Hirschsprung's disease (HSCR). One of the genes associated with HSCR is endothelin receptor type B ( Ednrb ). To study the development of colonic aganglionosis we have utilized a novel knockout mouse ( Ednrb flex3/flex3...

... to be of quail origin ( Fig. 5E-I ). † Author for correspondence (e-mail: A.Burns@ich.ucl.ac.uk ) * Present address: Stowers Institute for Medical Research, Kansas City, MO 64110, USA 7 3 2008 © 2008. 2008 Neural crest cell Enteric nervous system Gut Hirschsprung's disease...

... system, we removed β1 integrins in the neural crest cells when they emerge from the neural tube. β1-null enteric neural crest cells fail to colonise the gut completely, leading to an aganglionosis of the descending colon, which resembles the human Hirschsprung's disease. Moreover,β1-null enteric neural...

... progenitors of the enteric nervous system based on their ability to form distinct neurogenic and gliogenic structures in culture. Furthermore, these experiments provide the rationale for the development of novel approaches to the treatment of congenital megacolon (Hirschsprung's disease) based...

...Kirsten A. Dutton; Angela Pauliny; Susana S. Lopes; Stone Elworthy; Tom J. Carney; Jörg Rauch; Robert Geisler; Pascal Haffter; Robert N. Kelsh Waardenburg-Shah syndrome combines the reduced enteric nervous system characteristic of Hirschsprung’s disease with reduced pigment cell number, although...

...Raj P. Kapur; Cynthia Yost; Richard D. Palmiter ABSTRACT The lethal spotted ( ls ) mouse has been used as a model for the human disorder Hirschsprung’s disease, because as in the latter condition, ls/ls homozygotes are born without ganglion cells in their terminal colons and, without surgical...