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Heterogeneity in human iPSC-derived alveolar epithelial type II cells revealed with ABCA3/SFTPC reporters

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

ORGANISM(S): Homo sapiens

PROVIDER: GSE152657 | GEO | 2021/06/01

REPOSITORIES: GEO

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Heterogeneity in human iPSC-derived alveolar epithelial type II cells revealed with ABCA3/SFTPC reporters

Project description:Heterogeneity in human iPSC-derived alveolar epithelial type II cells revealed with ABCA3/SFTPC reporters

| PRJNA639964 | ENA

Global transcriptomic analyses of pluripotent stem cell-derived type II alveolar epithelial-like cells identified by ABCA3:GFP and SFTPCtdTomato bi-fluorescent reporters

Project description:We performed transcriptomic profiling of cells derived from human pluripotent stem cells (PSCs) using distal lung directed differentiation protocol previously described to generate alveolar type II epithelial-like cells (iAEC2s). We used the RUES2-STAG human embryonic stem cell line containing bi-fluorescently targeted ABCA3:GFP knock-in fusion reporter and SFTPCtdTomato knock-in reporter to potentially identify heterogenous iAEC2 populations and determine their gene expression profiles. On day 36 of differentiation, we sorted SFTPCtdTomato/ABCA3:GFP double positive (DP), ABCA3:GFP single positive (SP) and reporter double negative (DN) cells for transcriptomic analysis by bulk RNA sequencing. We found DN cells to be enriched in non-lung endodermal cells. Comparison between the reporter positive iAEC2 populations revealed high transcriptomic similarity with expression of canonical AEC2 transcripts in both the DP and SP cells. Analyses of differentially expressed transcript between the DP and SP cells revealed higher levels of surfactant and lamellar body-related transcripts in the DP cells. Moreover, we find enrichment of proliferative markers associated with cellular proliferation indicating the ABCA3:GFP single positive iAEC2s to potentially be a more proliferative subset of iAEC2s.

2021-06-01 | GSE152656 | GEO

Large ABCA3 and SFTPC deletions resulting in lung disease.

Project description:Mutations in genes encoding proteins important in the function and metabolism of pulmonary surfactant are recognized causes of lung disease. Clinical genetic testing is available for these disorders, but children with phenotypes consistent with surfactant dysfunction and no identifiable mutations in the known causative genes have been reported.To identify the mechanism(s) for lung disease in two children with the phenotype of surfactant dysfunction who had negative testing in clinical laboratories for gene mutations causing surfactant dysfunction.Amplicons spanning multiple exons of candidate genes were generated by polymerase chain reaction and sequenced.A 4,335-base deletion that included all of exon 12 of the gene encoding member A3 of the adenosine triphosphate-binding cassette transporter was identified in a full-term infant with respiratory failure. A 333-base deletion involving part of exon 4 and the adjacent intron of the gene encoding surfactant protein C was identified in a child with interstitial lung disease.Large deletions are a cause of surfactant dysfunction disorders and may need to be sought for specifically in children whose phenotypes suggest these syndromes but in whom clinical genetic testing is unrevealing.

| S-EPMC3960967 | biostudies-literature

Comparison of iPSC-derived alveolar epithelial cell culture methods (B2-3 SFTPC-GFP reporter iPSCs) and compound treatment in the alveolar epithelial spheroids (B2-3-dual-42-7 SFTPC-GFP AGER-mCherryHiBiT reporter iPSCs)

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

2024-02-29 | GSE241337 | GEO

Micro-patterned culture of iPSC-derived alveolar epithelial cells [alveolar]

Project description:We established a micro-patterned respiratory epithelial cell culture system in vitro. In this culture system, the iPSC-derived lung progenitor cells were differentiated into alveolar epithelial cells in a position-specific manner.

2024-02-29 | GSE236839 | GEO

Generation of ABCA3:GFP expressing type II alveolar epithelial-like cells from human pluripotent stem cells

Project description:We perform transcriptomic profiling of cells derived from human induced pluripotent stem cells (iPSCs) using distal lung directed differentiation protocol previously described to generate alveolar type II epithelial-like cells (iAEC2s). We use the BU3 ABCA3:GFP iPSC line containing a GFP knock-in fluorescent reporter for the canonical AEC2 gene, ABCA3, to sort out pure populations of GFP positive and negative cells on day 31 of distal lung differentiation for bulk RNA sequencing analysis. We find canonical AEC2s transcripts as well as lamellar body-associated transcripts to be enriched in the ABCA3:GFP positive population suggestive of enrichment of AEC2 program. In contrast, we find non-lung endodermal lineages to be enriched in the ABCA3:GFP negative cells. Combined, these findings indicate the reliability of the ABCA3:GFP reporter in enriching iAEC2s in our in vitro distal lung directed differentiation protocol.

2021-06-01 | GSE152500 | GEO

Alveolar injury and regeneration following deletion of ABCA3.

Project description:Adaptation to air breathing after birth is dependent upon the synthesis and secretion of pulmonary surfactant by alveolar type 2 (AT2) cells. Surfactant, a complex mixture of phospholipids and proteins, is secreted into the alveolus, where it reduces collapsing forces at the air-liquid interface to maintain lung volumes during the ventilatory cycle. ABCA3, an ATP-dependent Walker domain containing transport protein, is required for surfactant synthesis and lung function at birth. Mutations in ABCA3 cause severe surfactant deficiency and respiratory failure in newborn infants. We conditionally deleted the Abca3 gene in AT2 cells in the mature mouse lung. Loss of ABCA3 caused alveolar cell injury and respiratory failure. ABCA3-related lung dysfunction was associated with surfactant deficiency, inflammation, and alveolar-capillary leak. Extensive but incomplete deletion of ABCA3 caused alveolar injury and inflammation, and it initiated proliferation of progenitor cells, restoring ABCA3 expression, lung structure, and function. M2-like macrophages were recruited to sites of AT2 cell proliferation during the regenerative process and were present in lung tissue from patients with severe lung disease caused by mutations in ABCA3. The remarkable and selective regeneration of ABCA3-sufficient AT2 progenitor cells provides plausible approaches for future correction of ABCA3 and other genetic disorders associated with surfactant deficiency and acute interstitial lung disease.

| S-EPMC5752264 | biostudies-literature

CRISPR interference interrogation of COPD GWAS genes reveals the functional significance of desmoplakin in iPSC-derived alveolar epithelial cells.

Project description:Genome-wide association studies (GWAS) have identified dozens of loci associated with chronic obstructive pulmonary disease (COPD) susceptibility; however, the function of associated genes in the cell type(s) affected in disease remains poorly understood, partly due to a lack of cell models that recapitulate human alveolar biology. Here, we apply CRISPR interference to interrogate the function of nine genes implicated in COPD by GWAS in induced pluripotent stem cell-derived type 2 alveolar epithelial cells (iAT2s). We find that multiple genes implicated by GWAS affect iAT2 function, including differentiation potential, maturation, and/or proliferation. Detailed characterization of the GWAS gene DSP demonstrates that it regulates iAT2 cell-cell junctions, proliferation, mitochondrial function, and response to cigarette smoke-induced injury. Our approach thus elucidates the biological function, as well as disease-relevant consequences of dysfunction, of genes implicated in COPD by GWAS in type 2 alveolar epithelial cells.

| S-EPMC9278866 | biostudies-literature

Identification of early interstitial lung disease in an individual with genetic variations in ABCA3 and SFTPC.

Project description:A man with usual interstitial pneumonia (age of onset 58 years) was previously found to have an Ile73Thr (I73T) surfactant protein C (SFTPC) mutation. Genomic DNA from the individual and two daughters (aged 39 and 43 years) was sequenced for the I73T mutation and variations in ATP-binding cassette A3 (ABCA3). All three had the I73T SFTPC mutation. The father and one daughter (aged 39 years) also had a transversion encoding an Asp123Asn (D123N) substitution in ABCA3. The daughters were evaluated by pulmonary function testing and high-resolution CT (HRCT). Neither daughter had evidence of disease, except for focal subpleural septal thickening on HRCT scan in one daughter (aged 39 years). This daughter underwent bronchoscopy with transbronchial biopsies revealing interstitial fibrotic remodeling. These findings demonstrate that subclinical fibrotic changes may be present in family members of patients with SFTPC mutation-associated interstitial lung disease and suggest that ABCA3 variants could affect disease pathogenesis.

| S-EPMC2851554 | biostudies-other

Comparison of iPSC-derived alveolar epithelial cell culture methods

Project description:We established a novel alveolar epithelial culture method, called "On-Gel" culture. To characterize the "On-Gel" culture, we compared each transcriptome of the cultured cells in "On-Gel", fibroblast dependent-alveolar organoids (FD-AO) and fibroblast-free alveolar organoids (FF-AO) and their progenitor cells (CPMhigh Lung Progenitors).

2024-02-29 | GSE241335 | GEO

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