However, the development of HPS-FP disease is associated with severe surfactant accumulation, lysosomal stress, and AT2 cell apoptosis [144], and it has been found that spontaneous pulmonary fibrosis in double mutant Hps1/2 mice is associated with age, suggesting that pulmonary fibrosis with HPS gene mutation is associated with senescence of AT2 cells [93,94]. Here, HPS1 is linked to pulmonary fibrosis.