Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome pulmonary fibrosis (Updated March 9, 2024).

According to a preprint abstract from biorxiv.org, Hermansky-Pudlak syndrome (HPS) is a genetic disorder associated with pulmonary fibrosis. The study found that HPS mice exhibited a decline in alveolar epithelial type II (AT2) cell numbers with aging and abnormal differentiation, leading to an increase in AT2-derived alveolar epithelial type I cells. The researchers also discovered impaired AT2 cell proliferation and elevated expression of genes associated with aberrant differentiation and cellular senescence in HPS AT2 cells. These findings suggest that pulmonary fibrosis in HPS may be driven by AT2 cell progenitor dysfunction and p53-mediated senescence, providing a potential therapeutic target for HPS and other forms of pulmonary fibrosis. [Extracted from the article]