Epithelial-macrophage interactions determine pulmonary fibrosis susceptibility in Hermansky-Pudlak syndrome
Open accessAbstract
Alveolar epithelial cell (AEC) dysfunction underlies the pathogenesis of pulmonary fibrosis in Hermansky-Pudlak syndrome (HPS) and other genetic syndromes associated with interstitial lung disease; however, mechanisms linking AEC dysfunction and fibrotic remodeling are incompletely understood. Since increased macrophage recruitment precedes pulmonary fibrosis in HPS, we investigated whether crosstalk between AECs and macrophages determines fibrotic susceptibility. We found that AECs from HPS mice produce excessive MCP-1, which was associated with increased macrophages in the lungs of unchallenged HPS mice. Blocking MCP-1/CCR2 signaling in HPS mice with genetic deficiency of CCR2 or targeted deletion of MCP-1 in AECs normalized macrophage recruitment, decreased AEC apoptosis, and reduced lung fibrosis in these mice following treatment with low-dose bleomycin. We observed increased TGF-β production by HPS macrophages, which was eliminated by CCR2 deletion. Selective deletion of TGF-β in myeloid cells or of TGF-β signaling in AECs through deletion of TGFBR2 protected HPS mice from AEC apoptosis and bleomycin-induced fibrosis. Together, these data reveal a feedback loop in which increased MCP-1 production by dysfunctional AECs results in recruitment and activation of lung macrophages that produce TGF-β, thus amplifying the fibrotic cascade through AEC apoptosis and stimulation of fibrotic remodeling.
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The pathogenic role of Mo-AMs in lung fibrosis is largely attributed to their production of pro-fibrotic mediators such as TGF-β, PDGF-α, osteopontin, and MMPs.
“The pathogenic role of Mo-AMs in lung fibrosis has been largely attributed to their activity in producing a number of pro-fibrotic mediators, such as TGF-β, PDGF-α, osteopontin (OPN), and MMPs1,3,11,13,15,18,28.”
From TREM2 promotes lung fibrosis via controlling alveolar macrophage survival and pro-fibrotic activity (2025) · cites this paper as [28]
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DROSHA-Dependent miRNA and AIM2 Inflammasome Activation in Idiopathic Pulmonary Fibrosis
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Self-citationMatrix metalloproteinase activity in the lung is increased in Hermansky-Pudlak syndrome
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Macrophages in CRSwNP: Do they deserve more attention?
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Myeloid Fbxw7 Prevents Pulmonary Fibrosis by Suppressing TGF-β Production
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Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease
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Self-citationInsights into the Pathogenesis of Pulmonary Fibrosis from Genetic Diseases
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Self-citationLung injury and fibrosis induced by a mutant form of surfactant protein C
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Self-citationThe Interplay Between Innate Immunity and Nonimmune Cells in Lung Damage, Inflammation, and Repair
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Gene-edited MLE-15 Cells as a Model for the Hermansky-Pudlak Syndromes
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Self-citationAirway epithelial integrin β4‐deficiency exacerbates lipopolysaccharide‐induced acute lung injury
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IRAK-M Regulates Monocyte Trafficking to the Lungs in Response to Bleomycin Challenge
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Self-citationMechanisms and Therapeutic Potential of Myofibroblast Transformation in Pulmonary Fibrosis
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Fine-tuning the ubiquitin-proteasome system to treat pulmonary fibrosis
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Discoidin Domain Receptor 2, a Potential Therapeutic Target in Lung Fibrosis
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Dysregulated alveolar epithelial cell progenitor function and identity in Hermansky-Pudlak syndrome
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Self-citationCell-to-Cell Crosstalk: A New Insight into Pulmonary Hypertension
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Hermansky‐Pudlak Syndrome: From Molecular Pathogenesis to Targeted Therapies
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Hermansky-Pudlak syndrome: Gene therapy for pulmonary fibrosis
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Breaking Immunosuppressive Barriers by Engineered Nanoplatforms for Turning Cold Tumor to Hot
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Progressive pulmonary fibrosis in a murine model of Hermansky-Pudlak syndrome
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Self-citationPathogenesis and Therapy of Hermansky–Pudlak Syndrome (HPS)-Associated Pulmonary Fibrosis
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Self-citationImpairment of Renal Function in Hermansky-Pudlak Syndrome
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Fibrotic Signaling in the Lung
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Targeting CD30L Alleviates Airway Remodeling via JNK/p38 MAPK Pathway in OVA-Induced Asthmatic Mice
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Choline and CCL22 Are Prognostic Blood Biomarkers for Hermansky-Pudlak Syndrome Pulmonary Fibrosis
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The Role of Ninjurin1 on Pulmonary Fibrosis
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Is Immune Response Relevant in Interstitial Lung Disease?
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Self-citationAdditional file 3 of Progressive pulmonary fibrosis in a murine model of Hermansky-Pudlak syndrome
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- Authors
- Young, L. R., Gulleman, P. M., Short, C. W., Tanjore, H., Sherrill, T., Qi, A., McBride, A. P., Zaynagetdinov, R., Benjamin, J. T., Lawson, W. E., Novitskiy, S. V., & Blackwell, T. S.
- Affiliations
- Vanderbilt University (US)
- Department of Pediatrics, Division of Pulmonary Medicine, and
- Corresponding author
- Young, Lisa R.
- Venue
- JCI Insight
- Published
- 2016-10-20
- Volume
- 1
- Issue
- 17
- Pages
- e88947-e88947
- ISSN
- 2379-3708 (ISSN-L)
- PMC
- PMC5070955
- OpenAlex
- W2536886613
- Cited by
- 105
- References
- 51
- Citations by year
- 2026: 7 · 2025: 10 · 2024: 15 · 2023: 9 · 2022: 16 · 2021: 12 · 2020: 9 · 2019: 9 · 2018: 13 · 2017: 5
- FWCI
- 8.05
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- JCI Insight (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- U.S. Department of Veterans Affairs
- American Thoracic Society
- Hermansky-Pudlak Syndrome Network
- Vanderbilt University
- National Institutes of Health
- School of Medicine, Vanderbilt University
- National Heart, Lung, and Blood Institute (R01HL105479, RO1 HL, P01HL092870, R01HL119503, R01HL085317)
- National Cancer Institute (R01CA200681)
- National Institute of Child Health and Human Development
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (K12HD087023)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Keywords
- Hermansky-Pudlak syndrome, pulmonary fibrosis, alveolar epithelial cells, macrophage recruitment, MCP-1, CCR2, TGF-beta, TGFBR2, bleomycin-induced fibrosis, interstitial lung disease, myeloid cells
- MeSH terms
- Receptor, Transforming Growth Factor-beta Type II, Animals, Bleomycin, Disease Susceptibility, Epithelial Cells, Female, Macrophages, Male, Mice, Inbred C57BL, Pulmonary Alveoli, Pulmonary Fibrosis, Transforming Growth Factor beta, Protein Serine-Threonine Kinases, Receptors, Transforming Growth Factor beta, Chemokine CCL2, Hermanski-Pudlak Syndrome, Mice, Receptors, CCR2
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