ArticleNature communications2020
Conditional deletion of Nedd4-2 in lung epithelial cells causes progressive pulmonary fibrosis in adult mice.
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Article in Nature communications, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 54 papers.
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54citing papers in PubMed
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54 citing papers in PubMed, 105 citations in OpenAlex.
- Nedd4L-mediated stabilization of morphogenetic protein receptor 2 in lung fibroblasts confers protection against pulmonary fibrosis.Signal transduction and targeted therapy · 2026Article
- The Gut-Lung Microbiome Axis in Alveolar Stem Cell Regeneration and Lung Repair.Microorganisms · 2026Review
- Antifibrotic Drugs Regulate the Expression of Epithelial Sodium Channels in the Lungs.Advances in respiratory medicine · 2026Article
- NEDD4L-mediated Gasdermin D and E ubiquitination regulates cell death and tissue injury.Cell death and differentiation · 2026Article
- Decoding organ fibrosis: mechanistic insights and emerging therapeutic strategies.Signal transduction and targeted therapy · 2026Review
- Ferritinophagy-related prognostic genes UBE2Q1, NEDD4L, and TCP11L2 for prognosis prediction in sepsis.Scientific reports · 2025Article
- Review
- A novel human fetal lung-derived alveolar organoid model reveals mechanisms of surfactant protein C maturation relevant to interstitial lung disease.The EMBO journal · 2025Article
- Novel Integration of Spatial and Single-Cell Omics Data Sets Enables Deeper Insights into IPF Pathogenesis.Proteomes · 2025Article
- Ubiquitination Enzymes in Cancer, Cancer Immune Evasion, and Potential Therapeutic Opportunities.Cells · 2025Review
- Nedd4L signaling contributes to carbon tetrachloride-induced liver fibrosis in female mice and is associated with enteric dysbacteriosis.Gastroenterology report · 2025Article
- Targeting E3 ubiquitin ligases: a new frontier in idiopathic pulmonary fibrosis treatment.Frontiers in immunology · 2025Review
- Longitudinal microcomputed tomography detects onset and progression of pulmonary fibrosis in conditionalAmerican journal of physiology. Lung cellular and molecular physiology · 2024Article
- Therapeutic potential of omaveloxolone in counteracting muscle atrophy post-denervation: a multi-omics approach.Journal of translational medicine · 2024Article
- MFSD7C protects hemolysis-induced lung impairments by inhibiting ferroptosis.Nature communications · 2024Article
- Multiphase micro-computed tomography reconstructions provide dynamic respiratory function in a mouse lung fibrosis model.iScience · 2024Article
- circGRHPR inhibits aberrant epithelial-mesenchymal transformation progression of lung epithelial cells associated with idiopathic pulmonary fibrosis.Cell biology and toxicology · 2024Article
- Crosstalk of ubiquitin system and non-coding RNA in fibrosis.International journal of biological sciences · 2024Review
- microRNA-141-3p mediates epithelial cell proliferation, apoptosis, and epithelial-mesenchymal transition and alleviates pulmonary fibrosis in mice via Spred2.Histology and histopathology · 2023Article
- Review
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Authors and funding
23 authors at 7 institutions in 4 countries.
Julia Duerr *Department of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120, Heidelberg, Germany. julia.duerr@charite.de.ORCID http://orcid.org/0000-0002-4862-9487
Dominik H W Leitz *Department of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.
Magdalena Szczygiel *Translational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0002-5757-9370
Dmytro DvornikovTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.
Simon G FraumannDepartment of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.
Clemens KreutzInstitute of Medical Biometry and Statistics, University of Freiburg, Stefan-Meier-Straße 26, 79104, Freiburg, Germany.ORCID http://orcid.org/0000-0002-8796-5766
Piotr K ZadoraTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0001-9040-1227
Ayça Seyhan AgircanDepartment of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.
Philip KonietzkeTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0001-8580-6721
Theresa A EngelmannInstitute of Functional and Applied Anatomy, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Jan HegermannInstitute of Functional and Applied Anatomy, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Surafel MulugetaPulmonary, Allergy, and Critical Care Division, Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Boulevard, 19104, Philadelphia, PA, USA.
Hiroshi KawabeDepartment of Molecular Neurobiology, Max Planck Institute of Experimental Medicine, Hermann-Rein-Str. 3D, 37075, Goettingen, Germany.ORCID http://orcid.org/0000-0001-5650-8696
Lars KnudsenInstitute of Functional and Applied Anatomy, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
Matthias OchsInstitute of Functional and Applied Anatomy, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.ORCID http://orcid.org/0000-0002-0936-6979
Daniela RotinHospital for Sick Children and University of Toronto, 555 University Avenue, M5G 1X8, Toronto, Canada.ORCID http://orcid.org/0000-0001-5201-6280
Thomas MuleyTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0002-8141-0604
Michael KreuterTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0003-4402-2159
Felix J F HerthTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.
Mark O WielpützTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0001-6962-037X
Michael F BeersPulmonary, Allergy, and Critical Care Division, Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Boulevard, 19104, Philadelphia, PA, USA.ORCID http://orcid.org/0000-0002-2149-3209
Ursula KlingmüllerTranslational Lung Research Center (TLRC), German Center for Lung Research (DZL), Im Neuenheimer Feld 156, 69120, Heidelberg, Germany.ORCID http://orcid.org/0000-0001-9845-3099
Marcus A MallDepartment of Translational Pulmonology, University of Heidelberg, Im Neuenheimer Feld 156, 69120, Heidelberg, Germany. marcus.mall@charite.de.ORCID http://orcid.org/0000-0002-4057-2199
Heidelberg University · DEGerman Cancer Research Center · DEMedizinische Hochschule Hannover · DEUniversity of Pennsylvania · USMax Planck Institute of Experimental Medicine · DEUniversity of Freiburg · DEUniversity of Toronto · CA
Funding
Translational Research Support CoreP30ES013508 · NIEHS · UNIVERSITY OF PENNSYLVANIA · PI Trevor M Penning · 2006 to 2026
$35.3MTRAINING IN PULMONARY IMMUNOLOGYT32HL007586 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI MEYER, NUALA JENNINGS · 1985 to 2025
$8.8MAlveolar 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
$4.6MBiosynthesis and Trafficking of Surfactant Protein C In Health and DiseaseR01HL119436 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI BEERS, MICHAEL FRANCIS · 2013 to 2017
$2.0MSurfactant Protein C Mutations and Interstitial Lung DiseaseI01BX001176 · VA · PHILADELPHIA VA MEDICAL CENTER · PI MICHAEL FRANCIS BEERS · 2012 to 2026
–BLRD VA I01 BX001176CIHR MOP 130-422NHLBI NIH HHS R01 HL119436NHLBI NIH HHS R01 HL145408NHLBI NIH HHS T32 HL007586NIEHS NIH HHS P30 ES013508
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease characterized by patchy scarring of the distal lung with limited therapeutic options and poor prognosis. Here, we show that conditional deletion of the ubiquitin ligase Nedd4-2 (Nedd4l) in lung epithelial cells in adult mice produces chronic lung disease sharing key features with IPF including progressive fibrosis and bronchiolization with increased expression of Muc5b in peripheral airways, honeycombing and characteristic alterations in the lung proteome. NEDD4-2 is implicated in the regulation of the epithelial Na
Indexed as
AdultAgedAnimalsBiopsyDisease Models, AnimalEpithelial CellsEpithelial Sodium ChannelsHumansIdiopathic Pulmonary FibrosisLungMiceMice, KnockoutMiddle AgedMucin-5BNedd4 Ubiquitin Protein LigasesProteomicsEpithelial Sodium ChannelsMuc5b protein, mouseMucin-5BNedd4L protein, humanNedd4l protein, mouseNedd4 Ubiquitin Protein LigasespirfenidonePyridones
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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.