Cilia-dependent GLI processing in neural crest cells is required for tongue development.

ABSTRACT: Ciliopathies are a class of diseases caused by the loss of a ubiquitous, microtubule-based organelle called a primary cilium. Ciliopathies commonly result in defective development of the craniofacial complex, causing midfacial defects, craniosynostosis, micrognathia and aglossia. Herein, we explored how the conditional loss of primary cilia on neural crest cells (Kif3a^f/f;Wnt1-Cre) generated aglossia. On a cellular level, our data revealed that aglossia in Kif3a^f/f;Wnt1-Cre embryos was due to a loss of mesoderm-derived muscle precursors migrating into and surviving in the tongue anlage. To determine the molecular basis for this phenotype, we performed RNA-seq, in situ hybridization, qPCR and Western blot analyses. We found that transduction of the Sonic hedgehog (Shh)

SUBMITTER: Millington G 

PROVIDER: S-EPMC5420199 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature