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

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

PROVIDER: PRJNA1029617 | ENA |

REPOSITORIES: ENA

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			Action	DRS
	SRR23457569_1.fastq.gz	Fastqsanger.gz
	SRR23457569_2.fastq.gz	Fastqsanger.gz
	SRR23457570_1.fastq.gz	Fastqsanger.gz
	SRR23457570_2.fastq.gz	Fastqsanger.gz
	SRR23457571_1.fastq.gz	Fastqsanger.gz

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

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

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.

2025-01-09 | GSE245721 | GEO

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

Project description:Generation of expandable multipotential distal lung progenitors from human pluripotent stem cells that model idiopathic pulmonary fibrosis [bulk RNA-Seq]

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

Project description:This SuperSeries is composed of the SubSeries listed below.

2025-01-13 | GSE245723 | GEO

Transcriptome analysis of regional idiopathic pulmonary fibrosis airway epithelial cultures

Project description:We performed RNA sequencing of idiopathic pulmonary fibrosis (IPF) epithelia obtained from different regions of the disease lung and cultured at air-liquid interface. We analyzed cultures from large airways (>10 mm), small airways (<2 mm), non-fibrotic distal airways, fibrotic distal airways, and honeycombed regions.

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Idiopathic pulmonary fibrosis (IPF)

Project description:Idiopathic pulmonary fibrosis (IPF)

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Differential Lysotracker Uptake Defines Two Populations of Distal Epithelial Cells in Idiopathic Pulmonary Fibrosis

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Single cell RNA Sequencing Identifies G-protein Coupled Receptor 87 as a Basal Cell Marker Expressed in Distal Honeycomb Cysts in Idiopathic Pulmonary Fibrosis

Project description:Idiopathic Pulmonary Fibrosis (IPF) is a devastating and life-threatening lung disease characterized by epithelial reprogramming and increased extracellular matrix deposition leading to loss of lung function. A prominent histopathological structure in the IPF lung are aberrant bronchioles filled with mucus, referred to as honeycomb cysts. These heterogeneous bronchiolized areas feature clusters of simple epithelium with Keratin (KRT)5+ basal-like cells interspersed with pseudostratified epithelium containing differentiated, hyperplastic epithelial cells as well as aberrant ciliated cells. Recent single-cell RNA sequencing studies of the lung epithelium shed further light into cellular subtypes unique to IPF, including basaloid KRT5-/KRT17+ cells present in the distal lung. However, IPF distal bronchiole cell subtypes still remain poorly characterized and their disease contribution remains underinvestigated. Using single-cell RNA sequencing of enriched EpCAM+ lung cells of the distal IPF lung, we identified G-protein coupled receptor (GPR) 87, as a marker of distal bronchioles and KRT5+ IPF basal cells contributing to impaired airway differentiation and honeycombing.

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Idiopathic pulmonary fibrosis (IPF)

Project description:Idiopathic pulmonary fibrosis (IPF) is a disease related to AT2 cell. We used flow cytometry to analyze the epithelial component of donor and IPF lungs. From the live cells, we first excluded the CD31PosCD45Pos and then selected the EPCAMPos cells for further analysis using the human AT2 cell marker HTll-280 and the surface marker PD-L1. Our data indicate that, the bona fide differentiated AT2 cells (HTll-280High PD-L1Neg), were drastically reduced in the context of IPF. More interestingly, the number of HTll-280Low/Neg PD-L1High was drastically increased, suggesting that HTll-280Low PD-L1High epithelial cells could represent a pool of progenitors linked to the deficient AT2 lineage. The aim of this experiment is further characterization of AT2 and PDL1+ cells in donor and IPF.

2023-01-01 | GSE195770 | GEO

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