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Generation of expandable multipotential distal lung progenitors from human pluripotent stem cells that model idiopathic pulmonary fibrosis

ABSTRACT: This SuperSeries is composed of the SubSeries listed below.

ORGANISM(S): Homo sapiens

PROVIDER: GSE245723 | GEO | 2025/01/13

REPOSITORIES: GEO

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Generation of expandable multipotential distal lung progenitors from human pluripotent stem cells that model idiopathic pulmonary fibrosis [scRNA-Seq]

Project description:Human lungs contain unique cell populations in distal respiratory airways and terminal bronchioles (RA/TRB) that accumulate in patients with lung injury and idiopathic pulmonary fibrosis (IPF), a lethal lung disease. As these populations are absent in rodents, deeper understanding requires a human in vitro model. Here we convert human pluripotent stem cells into expandable spheres, called induced respiratory airway progenitors (iRAPs), consisting of ~99% RA/TRB-associated cell types. One hPSC can give rise to 1010 iRAP cells. We differentiate iRAPs through a stage consistent with transitional AT2 like cells into a population corresponding to mature AT1 cells with 95% purity. iRAPs with deletion of HPS1, which causes pulmonary fibrosis in humans, replicate the aberrant differentiation and recruitment of profibrotic fibroblasts observed in IPF, indicating that intrinsic dysfunction of RA/TRB-associated alveolar progenitors contributes to HPS1-related IPF. iRAPs may provide a system suitable for IPF drug discovery and validation.

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Project description:Human lungs contain distal respiratory airways (RAs) that are absent in rodents and contain unique cell populations that are more abundant in patients with lung injury and idiopathic pulmonary fibrosis (IPF). A deeper understanding of the potential of these cells requires an in vitro model. Here we report the generation from human pluripotent stem cells of expandable spheres (‘induced respiratory airway progenitors’ (iRAPs)) consisting of RA-associated cell types that could be differentiated into type 1 and type 2 alveolar epithelial cells in defined conditions and show defects that are hallmarks of IPF when mutant for HPS1, a gene associated with IPF in humans. These data indicate that alveolar epithelial cells can derive from distal airway progenitors and that their intrinsic dysfunction may be involved in aberrant, fibrotic repair in IPF. iRAPs thus provide a model to gain insight into human lung regeneration and into pathogenesis of IPF.

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