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Front Physiol. 2014 Feb 04;5:26. doi: 10.3389/fphys.2014.00026. eCollection 2014.

The emerging roles of ribosome biogenesis in craniofacial development.

Frontiers in physiology

Adam P Ross, Konstantinos S Zarbalis

Affiliations

  1. Department of Pathology and Laboratory Medicine, Institute for Pediatric Regenerative Medicine, Shriners Hospitals for Children, University of California at Davis Sacramento, CA, USA.

PMID: 24550838 PMCID: PMC3912750 DOI: 10.3389/fphys.2014.00026

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).

Keywords: TP53; cell cycle regulation; craniofacial development; neural crest; neurocristopathies; ribosome biogenesis; ribosomopathies

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