ArticleCell reports2021
Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease.
Registry-linked trialOpen access · goldAbstract read
In one paragraph
Article in Cell reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00014859 (Epidemiology of Surfactant Protein-B Deficiency), which is not on this map. Cited by 87 papers, 1 of them a synthesis that pooled it.
0numbers the graph read from it
0cells of the map it votes in
87citing papers in PubMed, 1 pooled it
10.0field-weighted citation impact, top 1% of its field
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Epidemiology of Surfactant Protein-B Deficiency
Who cites it
87 citing papers in PubMed, 1 synthesis or guideline pooled it, 107 citations in OpenAlex.
- The minor T allele of the MUC5B promoter rs35705950 associated with susceptibility to idiopathic pulmonary fibrosis: a meta-analysis.Scientific reports · 2021Pooled it
- Multi-omics insights into the mechanisms and prognosis of IPF.Genes and environment : the official journal of the Japanese Environmental Mutagen Society · 2026Review
- Lentiviral-mediated gene complementation to rescue pathogenic ABCA3 variants.American journal of respiratory cell and molecular biology · 2026Article
- Isoleucyl-tRNA synthetase 1 mutation impairs pulmonary surfactant homeostasis by disrupting alveolar macrophage function.Respiratory research · 2026Article
- Hallmarks of the ageing lung: 10 years later.The European respiratory journal · 2026Review
- Respiratory Organ-on-a-Chip for Disease Modeling: From Architecture to Functional Integration.Advanced healthcare materials · 2026Review
- Chronic TGFβ1 Signaling Drives Aberrant Alveolar-Basaloid Metaplasia through a KRT17-Stratifin migratory complex.bioRxiv : the preprint server for biology · 2026Article
- Macrophages orchestrate antiviral defense and epithelial repair in a human iPSC-derived alveolar air-liquid interface.JCI insight · 2026Article
- Long isoforms of the COPD risk gene FAM13A orchestrate human lung epithelial development.American journal of respiratory cell and molecular biology · 2026Article
- Unlocking Alveolar Regeneration: AT2 Stem Cells, Signaling Networks, and Therapeutic Frontiers.Stem cell reviews and reports · 2026Review
- AT2-intrinsic Z-AAT expression drives conserved inflammatory and proteotoxic stress responses and predisposes to emphysema.bioRxiv : the preprint server for biology · 2026Article
- Advancing dual gene therapy for SFTPC deficiency.Molecular therapy : the journal of the American Society of Gene Therapy · 2026Article
- Biallelic LAMP3 variants in 5 families with interstitial lung disease: Evidence of a disease-gene association.Genetics in medicine : official journal of the American College of Medical Genetics · 2026Article
- Syndecan-1 Promotes Alveolar Type 2 Epithelial Cell Senescence during Lung Fibrosis.bioRxiv : the preprint server for biology · 2026Article
- Efficient CRISPR-Cas RNP-based gene targeting of human AT2 cells.American journal of respiratory cell and molecular biology · 2026Article
- The chromatin remodeling complex PRC2 safeguards cell fate in alveolar epithelial type 2 cells.bioRxiv : the preprint server for biology · 2026Article
- Generation of an Induced Pluripotent Stem Cell-Derived Alveolar Type II In Vitro Model to Study Influenza A Virus Infection and Drug Treatments.Advanced healthcare materials · 2026Article
- Divergent pathways of surfactant protein C maturation for disease-associated isoforms.The Journal of biological chemistry · 2026Article
- Bidirectional fibrogenic cross-talk revealed in a human iPSC-derived epithelial-mesenchymal co-culture model of pulmonary fibrosis.bioRxiv : the preprint server for biology · 2026Article
- NAMs for closing knowledge gaps in assessment of respiratory uptake - a PARC project.Frontiers in toxicology · 2026Article
27 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
27 authors at 9 institutions in 1 country.
Konstantinos-Dionysios AlysandratosCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA; The Pulmonary Center and Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA.
Scott J RussoPulmonary, Allergy, and Critical Care Division, Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA; PENN-CHOP Lung Biology Institute, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Anton PetcherskiDepartments of Medicine, Endocrinology and Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Evan P TaddeoDepartments of Medicine, Endocrinology and Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Rebeca Acín-PérezDepartments of Medicine, Endocrinology and Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Carlos Villacorta-MartinCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA.
J C JeanCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA; The Pulmonary Center and Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA.
Surafel MulugetaPulmonary, Allergy, and Critical Care Division, Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA; PENN-CHOP Lung Biology Institute, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Luis R RodriguezPulmonary, Allergy, and Critical Care Division, Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA; PENN-CHOP Lung Biology Institute, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Benjamin C BlumDepartments of Biology and Biochemistry, Boston University School of Medicine, Boston, MA 02118, USA.
Ryan M HekmanDepartments of Biology and Biochemistry, Boston University School of Medicine, Boston, MA 02118, USA.
Olivia T HixCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA.
Kasey MinakinCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA.
Marall VedaieCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA; The Pulmonary Center and Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA.
Seunghyi KookDepartment of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University, Nashville, TN 37232, USA.
Andrew M Tilston-LunelDepartments of Biochemistry, Boston University School of Medicine, Boston, MA 02118, USA.
Xaralabos VarelasDepartments of Biochemistry, Boston University School of Medicine, Boston, MA 02118, USA.
Jennifer A WambachDivision of Newborn Medicine, Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, MO 63110, USA.
F Sessions ColeDivision of Newborn Medicine, Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, MO 63110, USA.
Aaron HamvasDepartment of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
Lisa R YoungDivision of Pulmonary and Sleep Medicine, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Marc LiesaDepartments of Medicine, Endocrinology and Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Andrew EmiliDepartments of Biology and Biochemistry, Boston University School of Medicine, Boston, MA 02118, USA.
Susan H GuttentagDepartment of Pediatrics, Monroe Carell Jr. Children's Hospital, Vanderbilt University, Nashville, TN 37232, USA.
Orian S ShirihaiDepartments of Medicine, Endocrinology and Molecular and Medical Pharmacology, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Michael F BeersPulmonary, Allergy, and Critical Care Division, Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA; PENN-CHOP Lung Biology Institute, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA. Electronic address: mfbeers@pennmedicine.upenn.edu.
Darrell N KottonCenter for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118, USA; The Pulmonary Center and Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA. Electronic address: dkotton@bu.edu.
Boston University · USBoston Medical Center · USUniversity of California, Los Angeles · USUniversity of Pennsylvania · USMallinckrodt (United States) · USChildren's Hospital of Philadelphia · USMonroe Carell Jr. Children's Hospital · USNorthwestern University · USVanderbilt University · US
Funding
Project-005UL1TR001430 · NCATS · BOSTON UNIVERSITY MEDICAL CAMPUS · PI BAIR-MERRITT, MEGAN H, CENTER, DAVID M. · 2015 to 2024
$52.3MTranslational Research Support CoreP30ES013508 · NIEHS · UNIVERSITY OF PENNSYLVANIA · PI Trevor M Penning · 2006 to 2026
$35.3MEditing 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
$7.5MAlveolar Epithelial Cell Dysfunction in Pulmonary Fibrosis: Leveraging SFTPC Mutations for Discovery of Molecular and Cellular TargetsR01HL145408 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI MICHAEL FRANCIS BEERS · 2019 to 2026
$4.6MA National iPS Cell Network with Deep Phenotyping for Translational ResearchU01TR001810 · NCATS · BOSTON UNIVERSITY MEDICAL CAMPUS · PI GILAD, YOAV, KOTTON, DARRELL N. · 2016 to 2020
$4.4MFunctional Characterization of ABCA3 Genomic VariantsR01HL149853 · NHLBI · WASHINGTON UNIVERSITY · PI Jennifer Wambach · 2020 to 2026
$3.9MSurfactant Protein C Mouse Models: A Fit For Purpose Preclinical Platform For Advancing Discovery In And Treatment Of Idiopathic Pulmonary FibrosisU01HL152970 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI BEERS, MICHAEL FRANCIS · 2021 to 2024
$3.0MNATIONAL BIOREPOSITORY OF LUNG DISEASE-SPECIFIC HUMAN INDUCED PLURIPOTENT STEM CELLS:THE PURPOSE OF THIS RESOURCE IS TO BUILD AND SUPPORT A CRITICAL75N92020C00005 · NHLBI · BOSTON UNIVERSITY MEDICAL CAMPUS · PI N/A, N/A · 2021 to 2024
$2.6MDeveloping a patient-specific organoid model of pulmonary fibrosis using iPSCsU01HL152976 · NHLBI · BOSTON UNIVERSITY MEDICAL CAMPUS · PI KOTTON, DARRELL N. · 2021 to 2024
$2.5MMetabolic reprogramming of Alveolar Type 2 cells in response to lung injuryR01HL150617 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI GUTTENTAG, SUSAN H. · 2022 to 2025
$2.4MBLRD VA I01 BX001176NCATS NIH HHS U01 TR001810NCATS NIH HHS UL1 TR001430NHLBI NIH HHS 75N92020C00005NHLBI NIH HHS K24 HL143281NHLBI NIH HHS R01 HL095993NHLBI NIH HHS R01 HL119436NHLBI NIH HHS R01 HL145408NHLBI NIH HHS R01 HL149853NHLBI NIH HHS R01 HL150617NHLBI NIH HHS U01 HL134745NHLBI NIH HHS U01 HL134766NHLBI NIH HHS U01 HL148692NHLBI NIH HHS U01 HL152970NHLBI NIH HHS U01 HL152976NIAAA NIH HHS R01 AA026914NIEHS NIH HHS P30 ES013508
Abstract
Alveolar epithelial type 2 cell (AEC2) dysfunction is implicated in the pathogenesis of adult and pediatric interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF); however, identification of disease-initiating mechanisms has been impeded by inability to access primary AEC2s early on. Here, we present a human in vitro model permitting investigation of epithelial-intrinsic events culminating in AEC2 dysfunction, using patient-specific induced pluripotent stem cells (iPSCs) carrying an AEC2-exclusive disease-associated variant (SFTPC
Indexed as
Alveolar Epithelial CellsAnimalsCell LineCell ProliferationEnergy MetabolismGenetic Predisposition to DiseaseHumansInduced Pluripotent Stem CellsInflammation MediatorsLung Diseases, InterstitialMiceMice, KnockoutMutationNF-kappa BPhenotypeProteostasisInflammation MediatorsNF-kappa BPulmonary Surfactant-Associated Protein CSFTPC protein, humanSftpc protein, mouseautophagybioenergeticsidiopathic pulmonary fibrosisinduced pluripotent stem cellsinterstitial lung diseaseiPSC-derived alveolar epithelial type 2 cellsmetabolic reprogrammingNF-κBproteostasissurfactant protein C
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LicenceCC BY-NC-ND
Read underepoch 390
Registered trials
NCT00014859ClinicalTrials.gov
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.