Abstract
Neural crest cells (NCCs) are a transient, migratory cell population, which originates during neurulation at the neural folds and contributes to the majority of tissues, including the mesenchymal structures of the craniofacial skeleton. The deregulation of the complex developmental processes that guide migration, proliferation, and differentiation of NCCs may result in a wide range of pathological conditions grouped together as neurocristopathies. Recently, due to their multipotent properties neural crest stem cells have received considerable attention as a possible source for stem cell based regenerative therapies. This exciting prospect underlines the need to further explore the developmental programs that guide NCC differentiation. This review explores the particular importance of ribosome biogenesis defects in this context since a specific interface between ribosomopathies and neurocristopathies exists as evidenced by disorders such as Treacher-Collins-Franceschetti syndrome (TCS) and Diamond-Blackfan anemia (DBA).
Highlights
Neural crest cells (NCCs) are a diverse population of migratory cells that give rise to most tissues during vertebrate development
Recent efforts have focused on the possibility of using neural crest-derived mesenchymal stem cells for regenerative purposes to address a plethora of degenerative disorders and injuries to tissues with limited capacity for repair and renewal
Disorders caused by defects in ribosome biogenesis, known as ribosomopathies, often affect neural crest-derived tissues posing interesting questions as to the particular significance this basic cellular function holds for the NCC lineage
Summary
The deregulation of the complex developmental processes that guide migration, proliferation, and differentiation of NCCs may result in a wide range of pathological conditions grouped together as neurocristopathies. Due to their multipotent properties neural crest stem cells have received considerable attention as a possible source for stem cell based regenerative therapies. This exciting prospect underlines the need to further explore the developmental programs that guide NCC differentiation.
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