Department of Biochemistry and Molecular Medicine, University of California at Davis, School of Medicine, Sacramento, CA 95817, USA.
Institute for Pediatric Regenerative Medicine of Shriners Hospitals for Children, University of California at Davis, School of Medicine, Sacramento, CA 95817, USA.
Dis Model Mech. 2019 Feb 4;12(2):dmm037051. doi: 10.1242/dmm.037051.
Diverse signaling cues and attendant proteins work together during organogenesis, including craniofacial development. Lip and palate formation starts as early as the fourth week of gestation in humans or embryonic day 9.5 in mice. Disruptions in these early events may cause serious consequences, such as orofacial clefts, mainly cleft lip and/or cleft palate. Morphogenetic Wnt signaling, along with other signaling pathways and transcription regulation mechanisms, plays crucial roles during embryonic development, yet the signaling mechanisms and interactions in lip and palate formation and fusion remain poorly understood. Various Wnt signaling and related genes have been associated with orofacial clefts. This Review discusses the role of Wnt signaling and its crosstalk with cell adhesion molecules, transcription factors, epigenetic regulators and other morphogenetic signaling pathways, including the Bmp, Fgf, Tgfβ, Shh and retinoic acid pathways, in orofacial clefts in humans and animal models, which may provide a better understanding of these disorders and could be applied towards prevention and treatments.
在器官发生过程中,包括颅面发育,各种信号线索和相关蛋白共同作用。人类的唇和腭裂形成最早始于妊娠第 4 周或小鼠的胚胎第 9.5 天。这些早期事件的中断可能会导致严重后果,如口面裂,主要是唇裂和/或腭裂。形态发生 Wnt 信号以及其他信号通路和转录调控机制在胚胎发育过程中起着至关重要的作用,但唇和腭裂形成和融合中的信号机制和相互作用仍知之甚少。各种 Wnt 信号及其相关基因与口面裂有关。这篇综述讨论了 Wnt 信号及其与细胞黏附分子、转录因子、表观遗传调控因子和其他形态发生信号通路(包括 Bmp、Fgf、Tgfβ、Shh 和视黄酸通路)的相互作用在人类和动物模型中的作用,这可能有助于更好地理解这些疾病,并可应用于预防和治疗。