2013
DOI: 10.1016/j.devcel.2013.05.021
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Abstract: SummaryCiliopathies are a broad class of human disorders with craniofacial dysmorphology as a common feature. Among these is high arched palate, a condition that affects speech and quality of life. Using the ciliopathic Fuz mutant mouse, we find that high arched palate does not, as commonly suggested, arise from midface hypoplasia. Rather, increased neural crest expands the maxillary primordia. In Fuz mutants, this phenotype stems from dysregulated Gli processing, which in turn results in excessive craniofacia… Show more

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Cited by 65 publications
(98 citation statements)
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“…A detailed developmental analysis presented in this study uncovered an enlarged maxillary process and aberrant patterns of NCC migration in E9.5 embryos. Interestingly, a NCC specific ablation of Fuzzy with Wnt1-Cre resulted in a true cleft phenotype without the associated maxillary hypoplasia or hypoplastic tongue (Tabler et al, 2013). Together, these findings are consistent with our model that cilia are actually performing discrete tasks in different regions of the embryo and future studies will need to incorporate this idea to fully explain the role of cilia in craniofacial NCCs.…”
Section: Insights From Animal Modelssupporting
confidence: 86%
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“…A detailed developmental analysis presented in this study uncovered an enlarged maxillary process and aberrant patterns of NCC migration in E9.5 embryos. Interestingly, a NCC specific ablation of Fuzzy with Wnt1-Cre resulted in a true cleft phenotype without the associated maxillary hypoplasia or hypoplastic tongue (Tabler et al, 2013). Together, these findings are consistent with our model that cilia are actually performing discrete tasks in different regions of the embryo and future studies will need to incorporate this idea to fully explain the role of cilia in craniofacial NCCs.…”
Section: Insights From Animal Modelssupporting
confidence: 86%
“…In these mutants, lineage tracing of NCCs indicates an expansion in the domain and number of NCCs in the midbrain, hindbrain, and maxillary process (Gray et al, 2009;Tabler et al, 2013). Taken together, these studies suggest a number of interesting possibilities regarding NCCs and primary cilia.…”
Section: Primary Cilia and Ncc Specificationmentioning
confidence: 76%
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“…This question seemed to pervade nearly every session of the workshop, and was addressed by a number of approaches. Building on previous studies (Tabler et al, 2013), Karen Liu (King's College London, UK) highlighted the essential role the environmental context plays in vivo by showing different populations of neural crest cells (NCCs) behave differently during migration. Tobias Zech (University of Liverpool, UK) presented a proteomics approach that identified differential protein interactions between 2D and 3D cultures, and provided evidence that 3D-specific adhesion sites are required for cell migration.…”
mentioning
confidence: 98%