Using Induced Pluripotent Stem Cells as a Tool to Understand Neurocristopathies

Neural crest cells (NCC) emerge from the neural plate border between the neural plate and non-neural ectoderm as the neural tube closes. These cells migrate throughout the early embryo, eventually contributing to a wide range of cell types, including bone, cartilage, adipose tissue, adrenal medulla, the peripheral nervous system and ganglia, melanocytes, and smooth muscle. Defects associated with the survival, migration, or differentiation of these cells underpins the development of a broad class of diseases known as neurocristopathies. The molecular basis for many neurocristopathies is poorly understood, in part because of limitations associated with the models used in their characterization. The recent development of induced pluripotent stem cell technology combined with the availability of patient-derived cells opens up new opportunities to address the molecular basis of neurocristopathies. This chapter discusses recent and potential future developments in this area.