ArticleStem cell reports2019
In Vitro Disease Modeling of Hermansky-Pudlak Syndrome Type 2 Using Human Induced Pluripotent Stem Cell-Derived Alveolar Organoids.
Article in Stem cell reports, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 49 papers.
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Who cites it
49 citing papers in PubMed, 98 citations in OpenAlex.
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- Microfluidic lung cancer models: Bridging clinical treatment strategies and tumor microenvironment recapitulation.APL bioengineering · 2025Review
- Modeling functional responses to pollutant exposure using modular hydrogel supported vascularized alveolosphere-on-a-chip.Biomaterials science · 2025Article
- A stem cell-based platform for functional analysis of genetic variants in lung disease.bioRxiv : the preprint server for biology · 2025Article
- Endogenous gene editing of alveolar organoids reveals that expression of pathogenic variant SFTPC-I73T disrupts endosomal function, epithelial polarity and wound healing.bioRxiv : the preprint server for biology · 2025Article
- Comprehensive characterization of human alveolar epithelial cells cultured for 28 days at the air-liquid interface.Scientific reports · 2025Article
- Evaluating the Use of iPSC-Derived Models in Understanding the Pathogenesis of Childhood Interstitial Lung Disease.Pediatric pulmonology · 2025Review
- Recent Advancements in the Generation and Application of Therapeutic Cell Populations for Lung Epithelial Repair.Journal of tissue engineering and regenerative medicine · 2025Review
- Human Pluripotent Stem Cell-Derived Alveolar Organoids for Gene-editing and Lung Adenocarcinomas Modeling.International journal of biological sciences · 2025Article
- Influence of mesenchymal and biophysical components on distal lung organoid differentiation.Stem cell research & therapy · 2024Article
- Building a human lung from pluripotent stem cells to model respiratory viral infections.Respiratory research · 2024Review
- Lung repair and regeneration: Advanced models and insights into human disease.Cell stem cell · 2024Review
- Human pluripotent stem cell modeling of alveolar type 2 cell dysfunction caused by ABCA3 mutations.The Journal of clinical investigation · 2024Article
- Human Pluripotent Stem Cell-Derived Alveolar Organoids: Cellular Heterogeneity and Maturity.Tuberculosis and respiratory diseases · 2024Article
- Recent advances in lung organoid development and applications in disease modeling.The Journal of clinical investigation · 2023Review
- Cryptotanshinone is a candidate therapeutic agent for interstitial lung disease associated with a BRICHOS-domain mutation ofiScience · 2023Article
- Modeling fibrotic alveolar transitional cells with pluripotent stem cell-derived alveolar organoids.Life science alliance · 2023Article
- Lack of racial and ethnic diversity in lung cancer cell lines contributes to lung cancer health disparities.Frontiers in oncology · 2023Review
Corrections and comments
- Erratum issued
Authors and funding
17 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
It has been challenging to generate in vitro models of alveolar lung diseases, as the stable culture of alveolar type 2 (AT2) cells has been difficult. Methods of generating and expanding AT2 cells derived from induced pluripotent stem cells (iPSCs) have been established and are expected to be applicable to disease modeling. Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder characterized by dysfunction of lysosome-related organelles, such as lamellar bodies (LBs), in AT2 cells. From an HPS type 2 (HPS2) patient, we established disease-specific iPSCs (HPS2-iPSCs) and their gene-corrected counterparts. By live cell imaging, the LB dynamics were visualized and altered distribution, enlargement, and impaired secretion of LBs were demonstrated in HPS2-iPSC-derived AT2 cells. These findings provide insight into the AT2 dysfunction in HPS patients and support the potential use of human iPSC-derived AT2 cells for future research on alveolar lung diseases.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.