Article

Modeling of fibrotic lung disease using 3D organoids derived from human pluripotent stem cells

2019-02-03

Abstract excerpt

Idiopathic pulmonary fibrosis (IPF) is an intractable interstitial lung disease for which no curative treatment is available except for lung transplantation. Its pathogenesis is unclear, but a role for injury to type 2 alveolar epithelial cells is hypothesized. Recessive mutations in some, but not all genes implicated in Hermansky-Pudlak Syndrome (HPS) cause HPS-associated interstitial pneumonia (HPSIP), a clinica...

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Literature Corpus work: 3a81787a-5a48-5072-b6b5-70ca9658bbeb
DOI: 10.1101/538991

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