Fuz mutant mice reveal shared mechanisms between ciliopathies and FGF-related syndromes.
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ABSTRACT:
SUBMITTER: Tabler JM
PROVIDER: S-EPMC3697100 | biostudies-literature | 2013 Jun
REPOSITORIES: biostudies-literature
Publications
Fuz mutant mice reveal shared mechanisms between ciliopathies and FGF-related syndromes.
Tabler Jacqueline M JM Barrell William B WB Szabo-Rogers Heather L HL Healy Christopher C Yeung Yvonne Y Perdiguero Elisa Gomez EG Schulz Christian C Yannakoudakis Basil Z BZ Mesbahi Aida A Wlodarczyk Bogdan B Geissmann Frederic F Finnell Richard H RH Wallingford John B JB Liu Karen J KJ
Developmental cell 20130601 6
Ciliopathies 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 cra ...[more]
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