Roxana Deleanu

By Roxana Nat, Galina Apostolova and Georg Dechant

Part of the book: Trends in Cell Signaling Pathways in Neuronal Fate Decision

By Roxana Nat, Andreas Eigentler and Georg Dechant

Part of the book: Pluripotent Stem Cells

By Roxana Nat

Intense research using vertebrate model organisms has gained considerable knowledge regarding the origin of peripheral neurons, such as neural crest and cranial placodes induction and diversification. However, early development in human embryos has remained largely uncharacterized, despite the roles the neural crest, cranial placodes and their derivatives play in several pathologies. The in vitro systems based on the differentiation of human pluripotent stem cells (hPSCs) strikingly recapitulate embryonic development in a dish. Extensively proved for the neurogenesis in the central nervous system (CNS) in the last 15 years, novel in vitro differentiation strategies were recently designed for the generation of peripheral nervous system (PNS)-related populations. It is the case of human neural crest, cranial placodes, cranial sensory and autonomic neurons, and enteric neurons. These novel models are equally important for enlightening the human early development and for developing new tools for the modern medicine. Better understanding of the programs for specification and maturation of the multitude of peripheral neurons is a major challenge confronting developmental and stem cell researchers in years to come.

Part of the book: Pluripotent Stem Cells

By Roxana Deleanu

The most affected cell types in cerebellar ataxias are the cerebellar neurons, which are not readily accessible for cellular and molecular investigation. Pluripotent stem cell (PSC) technology has emerged as an important tool for generating diverse types of neurons, which are used in order to better understand the human nervous system development and pathologies. In this chapter, the strategies for the differentiation of human PSCs toward cerebellar neurons are overviewed, followed by an outlook of their further optimization and diversification by implementing the knowledge from cerebellar development and new cell culture approaches. The optimization stategies are based on the recent progress made in defining the cell populations in mature and developing mouse and human cerebellum. The cellular phenotypes and organization in mouse and human cerebellum are briefly presented, followed by an overview of our current knowledge about their development, which includes pattering, proliferation, neurogenesis, gliogenesis, migration, connectivity and maturation. To date, however, relatively few studies have used induced PSCs (iPSCs) to model cerebellar ataxias and even fewer have looked directly to cerebellar neurons. The reported iPSC-derived in vitro models for cerebellar ataxias are reviewed, followed by an outlook of how to improve these models by generating and exporing the cerebellar neurons.

Part of the book: Spinocerebellar Ataxia