Loss of primary cilia leads to a decrease in PD-L1 expression, contributing to unchecked local T cell proliferation and activation in ciliopathies. This study reveals a connection between ciliary dysfunction and fibrosis in ciliopathic patients and animal models, suggesting a role for autoinflammation in disease onset. The loss of cilia in epithelial cells downregulates PD-L1 expression, promoting T cell activation. Furthermore, the study shows that primary cilia play a crucial role in maintaining PD-L1 expression and controlling T cell proliferation and activation. Understanding these mechanisms may lead to new treatment strategies for ciliopathies.
Source link