Bottasso-Arias Natalia, Mohanakrishnan Megha, Trovillion Sarah, Burra Kaulini, Russell Nicholas X, Wu Yixin, Xu Yan, Sinner Debora
Neonatology and Pulmonary Biology, Perinatal Institute. Cincinnati Children's Hospital Medical Center.
Neonatology and Pulmonary Biology Perinatal Institute. Cincinnati Children's Hospital Medical Center and University of Cincinnati Honors Program. Current affiliation University of Cincinnati, College of Medicine.
bioRxiv. 2024 Nov 4:2024.09.02.610014. doi: 10.1101/2024.09.02.610014.
The trachea is essential for proper airflow to the lungs for gas exchange. Frequent congenital tracheal malformations affect the cartilage, causing the collapse of the central airway during the respiratory cycle. We have shown that Notum, a Wnt ligand de-acylase that attenuates the canonical branch of the Wnt signaling pathway, is necessary for cartilaginous mesenchymal condensations. In Notum deficient tracheas, chondrogenesis is delayed, and the tracheal lumen is narrowed. It is unknown if Notum attenuates non-canonical Wnt signaling. We observed premature tracheal chondrogenesis after mesenchymal deletion of the non-canonical Wnt5a ligand. We hypothesize that Notum and Wnt5a are required to mediate the timely formation of mesenchymal condensations, giving rise to the tracheal cartilage. Ex vivo culture of tracheal tissue shows that chemical inhibition of the Wnt non-canonical pathway promotes earlier condensations, while Notum inhibition presents delayed condensations. Furthermore, non-canonical Wnt induction prevents the formation of cartilaginous mesenchymal condensations. On the other hand, cell-cell interactions among chondroblasts increase in the absence of mesenchymal Wnt5a. By performing an unbiased analysis of the gene expression in Wnt5a and Notum deficient tracheas, we detect that by E11.5, mRNA of genes essential for chondrogenesis and extracellular matrix formation are upregulated in Wnt5a mutants. The expression profile supports the premature and delayed chondrogenesis observed in Wnt5a and Notum deficient tracheas, respectively. We conclude that Notum and Wnt5a are necessary for proper tracheal cartilage patterning by coordinating timely chondrogenesis. Thus, these studies shed light on molecular mechanisms underlying congenital anomalies of the trachea.
气管对于肺部进行气体交换的正常气流至关重要。常见的先天性气管畸形会影响软骨,导致中央气道在呼吸周期中塌陷。我们已经表明,Notum是一种Wnt配体去酰基酶,可减弱Wnt信号通路的经典分支,它对于软骨间充质凝聚是必需的。在Notum缺陷的气管中,软骨形成延迟,气管腔变窄。尚不清楚Notum是否会减弱非经典Wnt信号。我们观察到在间充质中缺失非经典Wnt5a配体后气管软骨形成过早。我们假设Notum和Wnt5a是介导间充质凝聚及时形成所必需的,从而产生气管软骨。气管组织的体外培养表明,对Wnt非经典途径的化学抑制促进了更早的凝聚,而Notum抑制则导致凝聚延迟。此外,非经典Wnt诱导会阻止软骨间充质凝聚的形成。另一方面,在缺乏间充质Wnt5a的情况下,成软骨细胞之间的细胞间相互作用增加。通过对Wnt5a和Notum缺陷气管中的基因表达进行无偏分析,我们检测到在E11.5时,Wnt5a突变体中软骨形成和细胞外基质形成所必需的基因的mRNA上调。该表达谱分别支持了在Wnt5a和Notum缺陷气管中观察到的软骨形成过早和延迟。我们得出结论,Notum和Wnt5a通过协调及时的软骨形成对于正常的气管软骨模式是必需的。因此,这些研究揭示了气管先天性异常背后的分子机制。