Neural crest and Hirschsprung’s disease

Abstract

Neural crest cells are a transient population of stem cells in vertebrates that give rise to the entire peripheral nervous system (PNS) as well as various non-neural progenies. A peculiar control and coordination of proliferation, migration and differentiation is required for neural crest cells to generate a full diversity of progenies, navigate different organs and establish functional domains in their target organs. Defects in such developmental process may lead to a board spectrum of congenital disorders, and in some cases, also cancer. In this review, we will focus on one specific neurocristopathy in the PNS: the Hirschsprung’s disease (colonic aganglionosis), to emphasize how unraveling the molecular mechanisms underlying the neural crest cell fate determination and progression may facilitate our understanding of the disease etiologies and future development of therapies.