Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

Single cell transcriptomics in a treatment-segregated cohort exposes a STAT3-regulated therapeutic gap in idiopathic pulmonary fibrosis

The paper uses single-cell transcriptomics to analyze an IPF cohort, revealing a transcriptional deficit refractory to current treatments and identifying STAT3 as a key regulator in the therapeutic gap.

Wilms tumor 1 impairs apoptotic clearance of fibroblasts in distal fibrotic lung lesions

This study shows WT1 promotes fibroblast survival and ECM production in IPF by impairing apoptotic clearance.

Alveolar epithelial cell plasticity and injury memory in human pulmonary fibrosis

Single-nucleus multiomic and ex vivo lung slice profiling of human idiopathic pulmonary fibrosis identifies Alveolar Intermediate cells bridging alveolar and Aberrant Basaloid states and reveals AP-1 chromatin injury memory.

A Tunable Pulmonary Organoid Model Demonstrates Compositionally Driven Epithelial Plasticity and Immune Polarization

This paper introduces a tunable lung organoid model to study how cellular composition drives epithelial plasticity and immune polarization in chronic lung diseases.

Role of Arginine and its Metabolism in TGF-β-Induced Activation of Lung Fibroblasts

This study investigates the role of arginine and its metabolism in TGF-β-induced activation of lung fibroblasts, finding that arginine depletion inhibits mTORC1 activation and collagen production.

Clonal Expansion of Alveolar Fibroblast Progeny Drives Pulmonary Fibrosis

This study shows that fibroblast proliferation drives pulmonary fibrosis and that targeting this process could be a new therapeutic strategy.

Characteristics of ectopic alveolar basal cells relative to airway basal cells in fibrosis

The paper compares ectopic alveolar basal cells with airway basal cells in ILD patients, finding transcriptomic and organoid-forming differences but no evidence of AT2 cell origin.

SS-31 protects against bleomycin-induced lung injury and fibrosis

This study shows that SS-31 reduces bleomycin-induced lung fibrosis by protecting mitochondria, reducing oxidative stress, and lowering inflammation in mice.

PAI-1 Interaction with Sortilin Related Receptor-1 is Required for Lung Fibrosis

The paper identifies SorlA as a novel PAI-1 interactor required for lung fibrosis, showing SorlA deficiency protects against fibrosis in mice and SorlA is upregulated in human IPF lung tissue.

Resolving the molecular niche of pulmonary fibrosis using cryopreserved human precision cut lung slices

This study uses single-cell spatial transcriptomics on cryopreserved human lung slices to identify molecular features of pulmonary fibrosis niches and their associated signaling pathways.

β-III tubulin identifies anti-fibrotic state of pericytes in pulmonary fibrosis

This study identifies β-III tubulin as a marker for anti-fibrotic pericytes in pulmonary fibrosis and shows that Tuj1-expressing pericytes release CXCL10 to inhibit pro-fibrotic macrophage differentiation.

Direct M2 macrophage co-culture overrides viscoelastic hydrogel mechanics to promote fibroblast activation

This study shows that direct M2 macrophage co-culture promotes fibroblast activation regardless of hydrogel stiffness, mediated by IL6 signaling.

AXL-GAS6/PROS1 Interaction: A Critical Switch Between Aberrant- and Healthy Repair Following Alveolar Lung Injury

This study investigates ligand-specific AXL signaling in IPF, showing GAS6 promotes basal cell proliferation while PROS1 inhibits it, with implications for therapeutic targets.

Lung architecture amplifies tissue deposition in an agent-based model of fibrotic development

This study uses an agent-based model to simulate fibrotic development in the lung, finding that lung architecture enhances tissue deposition in fibrosis.

Accelerated Biological Aging in Familial Pulmonary Fibrosis

This study finds accelerated epigenetic aging in individuals at-risk for familial pulmonary fibrosis and patients, independent of telomere-related genetic variants or telomere length.

Contrasting responses of control and fibrotic lung fibroblasts to fibrotic stimuli: the role of osteoprotegerin in extracellular matrix remodeling

This study compares OPG secretion and ECM remodeling in control and fibrotic lung fibroblasts under fibrotic conditions, finding opposite responses in matrix stiffness and collagen fiber properties.

Biomaterial-based 3D human lung models replicate pathological characteristics of early pulmonary fibrosis

This paper develops 3D human lung models using biomaterials to replicate early pulmonary fibrosis features and tests drug responses.

YAP/TAZ are Crucial Regulator of Macrophage-mediated Pulmonary Inflammation and Fibrosis after Bleomycin-induced Injury

This study shows YAP/TAZ regulate macrophage behavior in pulmonary fibrosis, affecting inflammation, fibrosis, and tissue regeneration in mice.

Image-based spatial transcriptomics identifies molecular niche dysregulation associated with distal lung remodeling in pulmonary fibrosis

Xenium profiling of 343 genes in 1,126,820 nuclei from 19 human lungs maps fibrosis niches and orders alveolar remodeling from alveolar-capillary disruption to SPP1+ macrophage accumulation.

Ablation of LRP6 in alpha-smooth muscle actin-expressing cells abrogates lung inflammation and fibrosis upon bleomycin-induced lung injury

This study shows that deleting LRP6 in SMA-expressing cells prevents lung inflammation and fibrosis caused by bleomycin.

ATF4 and mTOR regulate metabolic reprogramming in TGF-β-treated lung fibroblasts

This study investigates how ATF4 and mTOR regulate metabolic reprogramming in TGF-β-treated lung fibroblasts, identifying their roles in amino acid homeostasis and glycolytic pathways.

JUNB O-GlcNAcylation-mediated promoter accessibility of metabolic genes modulates distinct epithelial lineage in pulmonary fibrosis

The paper identifies O-GlcNAcylation of JUNB as a regulator of promoter accessibility in metabolic genes, driving aberrant epithelial lineage in IPF.

Spatial multiomic profiling reveals distinct fibrotic epithelial niches in idiopathic pulmonary fibrosis

The paper uses spatial multiomic profiling to identify distinct fibrotic epithelial niches in IPF, revealing a KRT5low/KRT17 epithelial population and its association with SPP1 macrophages.

Interaction of osteoprotegerin with fibulin-1 is essential for extracellular matrix deposition in fibrotic lung tissue

This study investigates the interaction between osteoprotegerin and fibulin-1 in lung fibrosis, finding they form a complex with fibulin-1 bridging OPG and LTBP1, influencing extracellular matrix deposition.

Leukemia inhibitory factor (LIF) receptor amplifies pathogenic activation of fibroblasts in lung fibrosis

This study identifies LIFR as a master amplifier of fibroblast activation in IPF, showing that blocking LIFR reduces fibrosis in ex vivo tissue.

IFNγ-Expressing Myeloid Cells Localize within Lipoproteinosis during Drug-Associated Pulmonary Alveolar Proteinosis occurring in Systemic Juvenile Idiopathic Arthritis

This study identifies IFNγ-expressing myeloid cells in lipoproteinosis regions of sJIA-daPAP patients, suggesting their role in immune pathology.

A Novel Ex Vivo Approach for Investigating Profibrotic Macrophage Polarization Using Murine Precision-Cut Lung Slices

This paper develops an ex vivo model using murine precision-cut lung slices to study profibrotic macrophage polarization and validates its utility for fibrosis research.

Polygenic risk of idiopathic pulmonary fibrosis and COVID-19 severity

This study calculates polygenic risk scores for idiopathic pulmonary fibrosis (IPF) and finds they are associated with increased risk of severe COVID-19 outcomes, particularly in younger males.

Altered AP-1, RUNX, and EGR chromatin dynamics drive fibrotic lung disease

The paper identifies transcription factor networks driving fibrotic lung disease using multiomic single-cell data from SSc-ILD and control lungs.

Exploring the Antifibrotic Potential of the heparan sulfate mimetic OTR4120: Insights from Preclinical Models

This study evaluates the antifibrotic effects of OTR4120 in lung fibrosis models, showing reduced collagen deposition and fibroblast activation.