ArticleNature communications2025
Aberrant intermediate alveolar epithelial cells promote pathogenic activation of lung fibroblasts in preclinical fibrosis models.
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Article in Nature communications, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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29citing papers in PubMed
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Who cites it
29 citing papers in PubMed.
- Non-Resolving Repair in Idiopathic Pulmonary Fibrosis: From Failed Cellular Transitions to Architectural Lock-In.International journal of molecular sciences · 2026Review
- RNA Modifications as Molecular Regulators of Alveolar Epithelial Injury and Aberrant Repair in Pulmonary Fibrosis.Biomolecules · 2026Review
- Sustained YAP/TAZ activation promotes aberrant alveolar epithelial cell differentiation and drives persistent fibrotic remodeling.JCI insight · 2026Article
- Co-optation of the regenerative role of type 2 alveolar cells in distal lung repair in mice by terminal airway epithelial cells in humans.bioRxiv : the preprint server for biology · 2026Article
- Alveolar Epithelial Cell Loss of the Mitochondrial Regulator TFAM Drives Progressive Lung Fibrosis.bioRxiv : the preprint server for biology · 2026Article
- Targeting the fibrosis-inflammation-oxidative stress axis: multifaceted mechanisms of salidroside in chronic organ fibrosis.Apoptosis : an international journal on programmed cell death · 2026Review
- Murine models of pulmonary fibrosis: mechanisms, limitations and translational insights.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Ageing-associated regenerative failure in the lung: stem cell senescence and transitional cell persistence in idiopathic pulmonary fibrosis.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Integrative single-cell and bulk transcriptomic analyses identifyJournal of thoracic disease · 2026Article
- Epigenetic de-repression of basal cell metaplasia in aging AT2 cells is a risk factor for idiopathic pulmonary fibrosis (IPF).bioRxiv : the preprint server for biology · 2026Article
- Epithelial PCSK6 Promotes Proliferation and Decreases Collagen Deposition by Fibroblasts Potentially via MMP Activation.International journal of molecular sciences · 2026Article
- Fibroblasts as regulators of lung immunity, repair and fibrosis.Nature reviews. Immunology · 2026Review
- 2025 annual review of basic and translational research advances in pulmonary fibrosis: a narrative review.Journal of thoracic disease · 2026Review
- Disease Mechanisms and Therapeutic Advances in Idiopathic and Progressive Pulmonary Fibrosis: From Approved Drugs to Emerging Strategies.Journal of clinical medicine · 2026Review
- Chronic TGFβ1 Signaling Drives Aberrant Alveolar-Basaloid Metaplasia through a KRT17-Stratifin migratory complex.bioRxiv : the preprint server for biology · 2026Article
- Proof-of-Concept Evaluation of Primary Human FAP-CAR-NK Cells Targeting Activated Fibroblasts in Pulmonary Fibrosis.International journal of molecular sciences · 2026Article
- AT2-intrinsic Z-AAT expression drives conserved inflammatory and proteotoxic stress responses and predisposes to emphysema.bioRxiv : the preprint server for biology · 2026Article
- Hydrogel-Embedded Precision-Cut Lung Slices Recapitulate Fibrotic Gene Expression and Enable Therapeutic Response Evaluation.bioRxiv : the preprint server for biology · 2026Article
- Article
- Alveolar-Basal Intermediates Drive Pulmonary Fibrosis via Coordination of a Pro-Fibrotic Signaling Niche in Silicosis.bioRxiv : the preprint server for biology · 2026Article
Corrections and comments
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Authors and funding
24 authors.
Evan T Hoffman *Pulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Anit Shah *Pulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.ORCID http://orcid.org/0000-0002-6037-7585
Willy Roque BarbozaPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Luis R RodriguezPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.ORCID http://orcid.org/0000-0001-7176-0697
Rachna DherwaniPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Porter E DooleyCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.
Kasey MinakinCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.
Yaniv TomerPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Lauren J AyersCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.ORCID http://orcid.org/0000-0001-9236-7595
Dakota JonesCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.
Aditi MurthyPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Adam BennettPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Ana N LangeCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.
Pushpinder S BawaCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.
Feiya WangCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.
Apoorva BabuPENN -CHOP Lung Biology Institute, University of Pennsylvania, Philadelphia, PA, USA.
Katrina ChavezPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.ORCID http://orcid.org/0009-0000-2094-7275
Reilly S NakamotoPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Charlotte H CooperPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.
Maria C BasilPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA.ORCID http://orcid.org/0000-0003-4716-1285
Micha Sam Brickman RaredonDepartment of Anesthesiology, Yale School of Medicine, New Haven, CT, USA.ORCID http://orcid.org/0000-0003-1441-6122
Darrell N KottonCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.ORCID http://orcid.org/0000-0002-9604-8476
Konstantinos-Dionysios AlysandratosCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA, USA.ORCID http://orcid.org/0000-0002-5611-0343
Jeremy KatzenPulmonary, Allergy, and Critical Care Medicine Division, University of Pennsylvania, Philadelphia, PA, USA. Jeremy.Katzen@pennmedicine.upenn.edu.ORCID http://orcid.org/0000-0001-9208-7729
Funding
Patient-specific iPSCs to model and treat the inception of pulmonary fibrosisP01HL170952 · NHLBI · BOSTON UNIVERSITY MEDICAL CAMPUS · PI Darrell N. Kotton · 2024 to 2026
$11.4MTRAINING IN PULMONARY IMMUNOLOGYT32HL007586 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI MEYER, NUALA JENNINGS · 1985 to 2025
$8.8MEditing Alveolar Progenitor Cells for Correction of Monogenic DiseaseU01HL134745 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI KOTTON, DARRELL N., MORRISEY, EDWARD E · 2016 to 2022
$8.3MEpithelial stem/progenitor cells as repair agents in diffuse alveolar damageU01HL134766 · NHLBI · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI CHAPMAN, HAROLD A, KOTTON, DARRELL N. · 2016 to 2022
$8.1MDerivation of Transplantable Lung Epithelial Progenitors from iPS CellsR01HL095993 · NHLBI · BOSTON UNIVERSITY MEDICAL CAMPUS · PI Darrell N. Kotton · 2009 to 2026
$7.5MResearch Training in AnesthesiaT32GM086287 · NIGMS · YALE UNIVERSITY · PI ROBERT SCHONBERGER · 2009 to 2026
$3.7MDeveloping a patient-specific organoid model of pulmonary fibrosis using iPSCsU01HL152976 · NHLBI · BOSTON UNIVERSITY MEDICAL CAMPUS · PI KOTTON, DARRELL N. · 2021 to 2024
$2.5MAlveolar Epithelial Stress Signaling in Lung FibrosisR01HL172975 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI Jeremy Binder Katzen · 2025 to 2026
$1.4MUnderstanding how alveolar epithelial cell stress drives aberrant repair in interstitial lung diseaseK08HL150226 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI KATZEN, JEREMY BINDER · 2020 to 2024
$840kMolecular regulation of cell fate and progenitor function in the distal human respiratory airwaysK08HL163398 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI Maria Ciocca Basil · 2022 to 2026
$834kUtilizing induced pluripotent stem cells to study the role of alveolar type 2 cell dysfunction in pulmonary fibrosisK08HL163494 · NHLBI · BOSTON UNIVERSITY MEDICAL CAMPUS · PI Konstantinos Alysandratos · 2023 to 2026
$649kAging and Metabolic Dysfunction Drive Disrupted Alveolar Epithelial Progenitor Function in Pulmonary FibrosisK99HL171946 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI RODRIGUEZ, LUIS R · 2024 to 2024
$163kNHLBI NIH HHS K08 HL150226NHLBI NIH HHS K08 HL163398NHLBI NIH HHS K08 HL163494NHLBI NIH HHS K99 HL171946NHLBI NIH HHS P01 HL170952NHLBI NIH HHS R01 HL095993NHLBI NIH HHS R01 HL172975NHLBI NIH HHS T32 HL007586NHLBI NIH HHS U01 HL134745NHLBI NIH HHS U01 HL134766NHLBI NIH HHS U01 HL152976NIGMS NIH HHS T32 GM086287Pulmonary Fibrosis Foundation (PFF) Tully Family Familial Pulmonary Fibrosis AwardU.S. Department of Health & Human Services | NIH | National Heart, Lung, and Blood Institute (NHLBI) R01 HL172975-01A1
Abstract
Pulmonary fibrosis (PF) is a progressive disease histologically defined by pathological fibroblasts and epithelial cells. PF lungs contain alveolar type 2 epithelial cells (AT2s) that acquire an aberrant intermediate state phenotype. However, the direct role of these cells in PF and the signals causing them to arise and persist are not fully known. To address this, we harness the Sftpc
Indexed as
Alveolar Epithelial CellsFibroblastsLungPulmonary FibrosisAnimalsDisease Models, AnimalHumansInduced Pluripotent Stem CellsMiceMice, TransgenicMutationPulmonary Surfactant-Associated Protein CSignal TransductionTransforming Growth Factor betaPulmonary Surfactant-Associated Protein CSFTPC protein, humanSftpc protein, mouseTransforming Growth Factor beta
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LicenceCC BY-NC-ND
Read underepoch 390
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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.