Mouse models of Hirschsprung disease and other developmental disorders of the enteric nervous system: Old and new players.

ABSTRACT: Hirschsprung disease (HSCR, intestinal aganglionosis) is a multigenic disorder with variable penetrance and severity that has a general population incidence of 1/5000 live births. Studies using animal models have contributed to our understanding of the developmental origins of HSCR and the genetic complexity of this disease. This review summarizes recent progress in understanding control of enteric nervous system (ENS) development through analyses in mouse models. An overview of signaling pathways that have long been known to control the migration, proliferation and differentiation of enteric neural progenitors into and along the developing gut is provided as a framework for the latest information on factors that influence enteric ganglia formation and maintenance. Newly identified genes a

SUBMITTER: Bondurand N 

PROVIDER: S-EPMC5026931 | biostudies-literature | 2016 Sep

REPOSITORIES: biostudies-literature