Integration of IRF6 and Jagged2 signalling is essential for controlling palatal adhesion and fusion competence.
Integration of IRF6 and Jagged2 signalling is essential for controlling palatal adhesion and fusion competence.
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DOI：
10.1093/hmg/ddp201
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发表时间：
2009-07-15
影响因子：
3.5
通讯作者：
Dixon MJ
中科院分区：
文献类型：
--
作者：
Richardson RJ;Dixon J;Jiang R;Dixon MJ
在哺乳动物中，腭架的粘连和融合是继发性腭发育过程中的重要机制;这些过程的失败导致先天性异常，腭裂。防止口腔和腭上皮之间的病理性粘连，同时允许通过其内侧边缘上皮细胞的腭架的粘附和随后的融合的机制仍然不清楚。在人类中，转录因子干扰素调节因子6（IRF 6）的突变是货车德沃德综合征和腘翼状胬肉综合征的基础。最近，我们已经证明，小鼠纯合子突变Irf 6表现出异常的上皮分化，导致腭裂的结果之间的腭架和舌头的粘附。在目前的论文中，我们证明，IRF 6是必不可少的口腔上皮分化和IRF 6和Notch配体Jagged 2在收敛的分子途径在这个过程中的功能。我们进一步证明，IRF 6在口腔牙周膜的形成和维持中起着关键作用，时空调节对于确保适当的腭粘连至关重要。
In mammals, adhesion and fusion of the palatal shelves are essential mechanisms during the development of the secondary palate; failure of these processes leads to the congenital anomaly, cleft palate. The mechanisms that prevent pathological adhesion between the oral and palatal epithelia while permitting adhesion and subsequent fusion of the palatal shelves via their medial edge epithelia remain obscure. In humans, mutations in the transcription factor interferon regulatory factor 6 (IRF6) underlie Van der Woude syndrome and popliteal pterygium syndrome. Recently, we have demonstrated that mice homozygous for a mutation in Irf6 exhibit abnormalities of epithelial differentiation that results in cleft palate as a consequence of adhesion between the palatal shelves and the tongue. In the current paper, we demonstrate that Irf6 is essential for oral epithelial differentiation and that IRF6 and the Notch ligand Jagged2 function in convergent molecular pathways during this process. We further demonstrate that IRF6 plays a key role in the formation and maintenance of the oral periderm, spatio-temporal regulation of which is essential for ensuring appropriate palatal adhesion.
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影响因子：
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作者：
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通讯作者：
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