The crucial role of model systems in understanding the complexity of cell signaling in human neurocristopathies

Abstract

Animal models are useful to study the molecular, cellular and morphogenetic mechanisms underlying normal and pathological development. The neural crest (NC) is a transient, multipotent and migratory cell population that generates an astonishingly diverse array of cell types during vertebrate development. The abnormal formation or development of the NC results in neurocristopathies (NCP), which are characterized by a broad spectrum of functional and morphological alterations. The impaired molecular mechanisms that give rise to these multiphenotypic diseases are not entirely clear yet. This fact, added to the high incidence of these disorders in the newborn population, have led to the development of systematic approaches for their understanding. In this article, we systematically review the way in which experimentation with animal models has improved our knowledge of NCP, and how these advances might contribute to the development of better diagnostic and therapeutic tools for the treatment of these pathologies.