Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome
J. Y. Wang, N. S. Michki, S. Sitaraman, B. J. Banaschewski, R. Jamal, J. J. Gokey, S. M. Lin, J. B. Katzen, M. C. Basil, E. Cantu, J. A. Kropski, J. A. Zepp, D. B. Frank, L. R. Young
This study identifies intrinsic AT2 progenitor dysfunction and p53 pathway dysregulation as novel mechanisms driving pulmonary fibrosis in Hermansky-Pudlak syndrome.
bioRxiv · Jun 17, 2023Cell Biology