School and Hospital of Stomatology, Fujian Medical University, Fuzhou, Fujian, People's Republic of China.
Fujian Key Laboratory of Developmental and Neural Biology, College of Life Sciences, Fujian Normal University, Fuzhou, Fujian, People's Republic of China.
J Mol Histol. 2021 Aug;52(4):651-659. doi: 10.1007/s10735-021-09987-9. Epub 2021 Jun 2.
Tongue muscles are derived from mesodermal cells, while signals driven by cranial neural crest cells (CNCCs) regulate tongue myogenesis via tissue-tissue interaction. Based on such mechanisms of interaction, congenital tongue defects occur in CNC-related syndromes in humans. This study utilized a pathologic model for the syndrome of congenital bony syngnathia, Wnt1-Cre;pMes-Bmp4 mouse line, to explore impacts of enhanced CNCCs-originated BMP4 signal on tongue myogenesis via tissue-tissue interaction. Our results revealed that microglossia, a clinical phenotype of congenital bony syngnathia in humans exhibited in Wnt1-Cre;pMes-Bmp4 mice due to impaired myogenesis. The augmented BMP4 signal affected the distal distribution, proliferation, and differentiation of myogenic cells as well as tendon patterning, resulting in disarrangement and atrophy of tongue muscles and the loss of the anterior digastric muscle. This study demonstrated how a CNCCs-originated ligand impaired tongue myogenesis via a non-autonomous way, which provided potential formation mechanisms for understanding tongue abnormalities in CNC-related syndromes.
舌肌来源于中胚层细胞，而颅神经嵴细胞（CNCCs）发出的信号通过组织-组织相互作用调节舌肌发生。基于这种相互作用的机制，人类与 CNC 相关的综合征中会出现先天性舌缺陷。本研究利用先天性骨性并颌畸形综合征的病理模型，Wnt1-Cre;pMes-Bmp4 小鼠系，通过组织-组织相互作用，探讨增强的 CNCC 起源的 BMP4 信号对舌肌发生的影响。我们的结果表明，微舌症，即人类先天性骨性并颌畸形的一种临床表型，由于肌生成受损，在 Wnt1-Cre;pMes-Bmp4 小鼠中表现出来。增强的 BMP4 信号影响了肌源性细胞的远端分布、增殖和分化以及肌腱模式形成，导致舌肌排列紊乱和萎缩，以及前二腹肌的丧失。本研究展示了 CNCC 起源的配体如何通过非自主方式损害舌肌发生，为理解 CNC 相关综合征中的舌异常提供了潜在的形成机制。
