Cell BiologybioRxiv
Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome
J. Y. Wang, N. S. Michki, S. Sitaraman…·L. R. Young
This study identifies intrinsic AT2 progenitor dysfunction and p53 pathway dysregulation as novel mechanisms driving pulmonary fibrosis in Hermansky-Pudlak syndrome.
DOI 10.1172/jci.insight.183483