Zhu Xiao-Jing, Liu Yudong, Yuan Xueyan, Wang Min, Zhao Wanxin, Yang Xueqin, Zhang Xiaoyun, Hsu Wei, Qiu Mengsheng, Zhang Ze, Zhang Zunyi
Institute of Developmental and Regenerative Biology, College of Life and Environmental Science, Hangzhou Normal University, Zhejiang, China.
Department of Biomedical Genetics, Center for Oral Biology, James P Wilmot Cancer Center, University of Rochester Medical Center, Rochester, New York.
Dev Dyn. 2016 Mar;245(3):414-26. doi: 10.1002/dvdy.24376. Epub 2016 Jan 8.
Mutations of WNT3, WNT5A, WNT9B, and WNT11 genes are associated with orofacial birth defects, including nonsyndromic cleft lip with cleft palate in humans. However, the source of Wnt ligands and their signaling effects on the orofacial morphogenetic process remain elusive.
Using Foxg1-Cre to impair Wnt secretion through the inactivation of Gpr177/mWls, we investigate the relevant regulation of Wnt production and signaling in nasal-facial development. Ectodermal ablation of Gpr177 leads to severe facial deformities resulting from dramatically reduced cell proliferation and increased cell death due to a combined loss of WNT, FGF and BMP signaling in the developing facial prominence. In the invaginating nasal pit, the Gpr177 disruption also causes a detrimental effect on migration of the olfactory epithelial cells into the mesenchymal region. The blockage of Wnt secretion apparently impairs the olfactory epithelial cells through modulation of JNK signaling.
Our study thus suggests the head ectoderm, including the facial ectoderm and the neuroectoderm, as the source of canonical as well as noncanonical Wnt ligands during early development of the nasal-facial prominence. Both β-catenin-dependent and -independent signaling pathways are required for proper development of these morphogenetic processes.
WNT3、WNT5A、WNT9B和WNT11基因的突变与口面部出生缺陷相关,包括人类非综合征性唇腭裂。然而,Wnt配体的来源及其对口面部形态发生过程的信号传导作用仍不清楚。
利用Foxg1-Cre通过使Gpr177/mWls失活来损害Wnt分泌,我们研究了鼻面部发育中Wnt产生和信号传导的相关调节。Gpr177的外胚层消融导致严重的面部畸形,这是由于发育中的面部隆起中WNT、FGF和BMP信号的联合丧失导致细胞增殖显著减少和细胞死亡增加所致。在正在内陷的鼻凹中,Gpr177的破坏也对嗅觉上皮细胞向间充质区域的迁移产生不利影响。Wnt分泌的阻断显然通过调节JNK信号传导损害嗅觉上皮细胞。
因此,我们的研究表明,在鼻面部隆起的早期发育过程中,头部外胚层,包括面部外胚层和神经外胚层,是经典和非经典Wnt配体的来源。β-连环蛋白依赖性和非依赖性信号通路对于这些形态发生过程的正常发育都是必需的。