关键词: cleft palate/lip congenital disorder genetic network palate development

Mesh : Mice Animals Humans Cleft Palate / genetics Gene Regulatory Networks Quality of Life Hedgehog Proteins / genetics Wnt Signaling Pathway / genetics Fibroblast Growth Factors / genetics

来  源:   DOI:10.3390/cells12151954   PDF(Pubmed)

Abstract:
Palatogenesis is a complex and intricate process involving the formation of the palate through various morphogenetic events highly dependent on the surrounding context. These events comprise outgrowth of palatal shelves from embryonic maxillary prominences, their elevation from a vertical to a horizontal position above the tongue, and their subsequent adhesion and fusion at the midline to separate oral and nasal cavities. Disruptions in any of these processes can result in cleft palate, a common congenital abnormality that significantly affects patient\'s quality of life, despite surgical intervention. Although many genes involved in palatogenesis have been identified through studies on genetically modified mice and human genetics, the precise roles of these genes and their products in signaling networks that regulate palatogenesis remain elusive. Recent investigations have revealed that palatal shelf growth, patterning, adhesion, and fusion are intricately regulated by numerous transcription factors and signaling pathways, including Sonic hedgehog (Shh), bone morphogenetic protein (Bmp), fibroblast growth factor (Fgf), transforming growth factor beta (Tgf-β), Wnt signaling, and others. These studies have also identified a significant number of genes that are essential for palate development. Integrated information from these studies offers novel insights into gene regulatory networks and dynamic cellular processes underlying palatal shelf elevation, contact, and fusion, deepening our understanding of palatogenesis, and facilitating the development of more efficacious treatments for cleft palate.
摘要:
腭生是一个复杂而复杂的过程,涉及通过各种形态发生事件形成腭,高度依赖于周围环境。这些事件包括从胚胎上颌突出处生长出的腭架,它们从舌头上方的垂直位置到水平位置的高度,以及它们随后在中线的粘附和融合,以分离口腔和鼻腔。这些过程中的任何一个中断都会导致腭裂,一种常见的先天性异常,显著影响患者的生活质量,尽管有手术干预。尽管通过对转基因小鼠和人类遗传学的研究已经确定了许多参与腭生成的基因,这些基因及其产物在调节腭突发生的信号网络中的确切作用仍然难以捉摸。最近的调查显示,腭架生长,图案化,附着力,和融合受到众多转录因子和信号通路的复杂调节,包括刺猬(Shh),骨形态发生蛋白(Bmp),成纤维细胞生长因子(Fgf),转化生长因子β(Tgf-β),Wnt信号,和其他人。这些研究还确定了大量对腭发育至关重要的基因。来自这些研究的综合信息为基因调控网络和pal架抬高的动态细胞过程提供了新的见解,联系人,和融合,加深我们对腭发生的理解,并促进开发更有效的腭裂治疗方法。
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