Ablation of the Sox11 Gene Results in Clefting of the Secondary Palate Resembling the Pierre Robin Sequence.

The Journal of Biological Chemistry
Huarong HuangZunyi Zhang

Abstract

Mouse gene inactivation has shown that the transcription factor Sox11 is required for mouse palatogenesis. However, whether Sox11 is primarily involved in the regulation of palatogenesis still remains elusive. In this study, we explored the role ofSox11in palatogenesis by analyzing the developmental mechanism in cleft palate formation in mutants deficient in Sox11. Sox11 is expressed both in the developing palatal shelf and in the surrounding structures, including the mandible. We found that cleft palate occurs only in the mutant in which Sox11is directly deleted. As in the wild type, the palatal shelves in the Sox11 mutant undergo outgrowth in a downward direction and exhibit potential for fusion and elevation. However, mutant palatal shelves encounter clefting, which is associated with a malpositioned tongue that results in physical obstruction of palatal shelf elevation at embryonic day 14.5 (E14.5). We found that loss of Sox11led to reduced cell proliferation in the developing mandibular mesenchyme via Cyclin D1, leading to mandibular hypoplasia, which blocks tongue descent. Extensive analyses of gene expression inSox11 deficiency identified FGF9 as a potential candidate target of Sox11 in the modulation of cell proliferati...Continue Reading

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Citations

Apr 14, 2017·American Journal of Medical Genetics. Part a·Srinivas M SusarlaKelly N Evans
Apr 2, 2017·Developmental Biology·Joan E HooperTrevor Williams
Feb 14, 2018·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Umair KhanJill Clayton-Smith
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Jul 16, 2020·Birth Defects Research·Kurt ReynoldsChengji J Zhou
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Mar 5, 2021·Biochemical and Biophysical Research Communications·Satoshi NunomuraKenji Izuhara
Apr 15, 2018·Biomedicine & Pharmacotherapy = Biomédecine & Pharmacothérapie·Mengjia WengZhenqi Chen

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