Fig. 8
From: PKD1 deficiency induces Bronchiectasis in a porcine ADPKD model
A simplified model illustrating the role ofPKD1in bronchiectasis. In PKD1+/− pig airway cells, PKD1 deficiency caused E-cadherin downregulated, which leaded to epithelial barrier dysfunction. Along with the acceleration of harmful substance invasion and increase inflammation response, the components of airway extracellular matrix (MMP9, MMP1, Collagen IV and Collagen I) were altered, which in turn upregulated ERK signaling and inhibited ROCK signaling in airway smooth muscle cells, leading to the phenotype switch from contractile phenotype to proliferative phenotype. The increased dysfunctional airway smooth muscle resulted in the remodeling of airway and bronchiectasis