Article

Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome

2023-06-18

Abstract excerpt

Hermansky-Pudlak syndrome (HPS) is a genetic disorder of endosomal protein trafficking associated with pulmonary fibrosis in specific subtypes, including HPS-1 and HPS-2. Single mutant HPS1 and HPS2 mice display increased fibrotic sensitivity while double mutant HPS1/2 mice exhibit spontaneous fibrosis with aging, which has been attributed to HPS mutations in alveolar epithelial type II (AT2) cells. We utilized HP...

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Literature Corpus work: 570d438b-e003-50aa-a134-600330d5b9d7
DOI: 10.1101/2023.06.17.545390

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