Cystic fibrosis

Macrophage-derived glycoprotein nonmetastatic melanoma protein B (GPNMB) is trapped by fibrotic extracellular matrix in a pulmonary fibrosis model and may activate resident normal fibroblasts, promoting fibrosis.

Patients with cystic fibrosis are not reporting particularly sever outcomes upon SARS-CoV-2 infection. Here, the authors demonstrate decreased ACE2 levels is cystic fibrosis airway epithelia associated with impaired viral entry and replication.

Currently, there is a lack of effective drugs for the treatment of pulmonary fibrosis. Here, the authors reveal a novel role of PEAR1 in fibroblast activation and demonstrate that activating PEAR1 by monoclonal antibodies might be a promising therapeutic approach for pulmonary fibrosis.