Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

Modeling cell-cell interactions to advance drug discovery in Idiopathic Pulmonary Fibrosis

This study develops a 3D co-culture organoid model of IPF to investigate epithelial-fibroblast interactions and test anti-fibrotic drugs.

TP53-mediated bidirectional lineage plasticity drives alveolar epithelial cell extrusion and tissue remodeling

The paper identifies TP53 activation in alveolar epithelial cells as a driver of cell extrusion and tissue remodeling in IPF through bidirectional lineage plasticity.

Systematic Quantitative Proteomics Defines Age, Sex, and Region-Dependent Remodeling of Lung Extracellular Matrix

This study uses proteomics to show that aging, sex, and region affect lung ECM remodeling, with age being the main factor and females showing more pronounced changes.

Trajectory inference of epithelial-centered neighborhood profiles reconstructs a pseudo-temporal continuum in idiopathic pulmonary fibrosis

The paper reconstructs a pseudo-temporal continuum of fibrotic remodeling in IPF by analyzing epithelial-centered neighborhood profiles across spatially heterogeneous lung tissue.

Epigenetic de-repression of basal cell metaplasia in aging AT2 cells is a risk factor for idiopathic pulmonary fibrosis (IPF).

This study identifies age-related epigenetic changes in AT2 cells that promote basal cell metaplasia, contributing to idiopathic pulmonary fibrosis.

Early Pulmonary Fibrosis is Defined by Niche- and Cell-Specific Molecular Programs

This paper identifies compartment- and cell-specific molecular programs in preclinical familial pulmonary fibrosis using multi-omic profiling and finds these signatures are partially represented in peripheral blood.

Toll-Like-Receptor 5 protects against pulmonary fibrosis by reducing lung dysbiosis

TLR5 deficiency is linked to pulmonary fibrosis due to its role in preventing microbial dysbiosis following lung injury.

Latent transforming growth factor binding protein-2 (LTBP2), an IPF biomarker of clinical decline, promotes TGF-beta signaling and lung fibrosis in mice

This study shows LTBP2 promotes TGF-beta signaling and lung fibrosis in mice but is also needed for epithelial repair.

O-GlcNAc transferase regulates H2O2 production via p38 MAPK

This paper shows that OGT inhibition reduces TGF-β-induced p38 MAPK signaling and H2O2 production in lung fibroblasts through O-GlcNAcylation of p38 and p47phox.

Alveolar niche disruption and aberrant epithelial reprogramming are early hallmarks of idiopathic pulmonary fibrosis

This study identifies early alveolar niche disruption and aberrant epithelial reprogramming in IPF using multi-modal analyses of patient cohorts.

Genome-wide association study of Idiopathic Pulmonary Fibrosis susceptibility using clinically-curated European-ancestry datasets

This study identifies three novel genetic signals associated with idiopathic pulmonary fibrosis using GWAS and clinically-curated datasets, highlighting potential drug targets like MUC1 and NTN4.

Rare bi-allelic loss-of-function variants in the LRRK2 kinase cause interstitial lung disease

This study identifies biallelic LRRK2 loss-of-function variants as a cause of interstitial lung disease, showing reduced LRRK2 protein and impaired lung function in affected individuals.

Proteomic analysis reveals regional sex differences in healthy and fibrotic human lung

This study identifies sex-biased protein expression in healthy and fibrotic human lung regions, highlighting potential biological mechanisms for sex differences in idiopathic pulmonary fibrosis.

Differential collagen crosslinking and network organization creates distinct tissue remodeling patterns in fibrosis and COPD

The paper examines collagen crosslinking and organization in healthy and diseased human lungs, finding differences in crosslinking and collagen maturity between fibrotic and COPD tissues.

Engineering a 3D Lung Co-culture Platform to Model Epithelial-Fibroblast Interactions in Pulmonary Fibrosis

This paper develops a 3D lung co-culture model to simulate epithelial-fibroblast interactions in IPF, showing it captures key fibrotic processes like myofibroblast differentiation and collagen disorganization.

Collagen crosslinking and organizational patterns reflect common disease processes in idiopathic pulmonary fibrosis and non-resolving acute respiratory distress syndrome

The paper compares collagen organization and crosslinking in IPF and NR-ARDS, finding differences in collagen maturity despite similar collagen content.

The pleuroparenchymal fibroelastosis atlas reveals aberrant cell states and their zonation as an alternate roadmap to lung fibrosis

This paper presents the first single-nucleus RNA sequencing atlas of pleuroparenchymal fibroelastosis, identifying novel cell states and their spatial organization in lung fibrosis.

An agent-based model suggests how senescent cell behavior and matrix mechanics drive pulmonary fibrosis in aged mice

The paper develops an agent-based model to show how senescent cell behavior and tissue mechanics contribute to pulmonary fibrosis in aged mice.

Cross-ancestry evaluation of idiopathic pulmonary fibrosis genetic risk variants

This study evaluates the genetic risk variants for idiopathic pulmonary fibrosis in non-European populations, finding partial replication and ancestry-specific effects, with rs35705950 as the most consistent signal.

TGF-β Coordinates Alanine Synthesis and Import for Myofibroblast Differentiation in Pulmonary Fibrosis

This study identifies alanine as a critical metabolite regulated by TGF-β that supports myofibroblast differentiation in pulmonary fibrosis through GPT2 and SLC38A2.

Polygenic risk scores enhance the identification of carriers of monogenic forms of idiopathic pulmonary fibrosis

This study shows that polygenic risk scores improve the identification of IPF patients carrying rare variants associated with monogenic pulmonary fibrosis.

Transcriptional and spatial profiling of fibroblasts from human lungs highlights CTHRC1+ cells as fibrogenic signaling hubs in fibrosis

The paper integrates single-cell RNA sequencing and spatial transcriptomics to identify CTHRC1+ fibroblasts as key fibrogenic cells in IPF, showing how culture affects their transcriptional profile.

Myeloperoxidase promotes fibrosis by inhibiting cathepsin K to bias the lung toward ECM accumulation

This study identifies MPO as a post-translational inhibitor of CatK, linking acute inflammation to sustained fibrosis through impaired collagen degradation.

Longitudinal Single-Cell RNA-seq Profiling of Lung Cell Phenotypes, Signaling, and Cross-talk During Fibrosis Resolution

The paper uses longitudinal single-cell RNA-seq to study lung fibrosis resolution, identifying pro- and anti-fibrotic pathways and cellular interactions during recovery.

Dysplastic Epithelial Repair Propagates Chronic Pathology Through the Paracrine Transformation of Pulmonary Fibroblasts

The paper shows dysplastic basal cells induce an inflammatory fibroblast phenotype through IL-1 secretion, promoting chronic inflammation and fibrosis.

Targeting the DNA damage repair protein RAD51 alters fibroblast metabolism and enhances apoptosis in pulmonary fibrosis

This study shows that inhibiting RAD51 in fibroblasts reduces fibrosis by promoting apoptosis and metabolic changes.

Syndecan-1 Promotes Alveolar Type 2 Epithelial Cell Senescence during Lung Fibrosis.

This study shows that syndecan-1 promotes AT2 cell senescence in lung fibrosis, contributing to fibrotic remodeling and suggesting it as a therapeutic target.

Hydrogel-Embedded Precision-Cut Lung Slices Recapitulate Fibrotic Gene Expression and Enable Therapeutic Response Evaluation

This paper develops a hydrogel-embedded PCLS model to study pulmonary fibrosis, showing prolonged viability and matching fibrotic gene expression patterns in human tissue.

Clonal Hematopoiesis Instructs Maladaptive Tissue Repair to Promote Fibrosis

This study shows clonal hematopoiesis promotes fibrosis by altering macrophage behavior and tissue repair processes in lung disease.

Agent-Based Modeling of Idiopathic Lung Fibrosis and Mechanistic Treatments

This paper uses agent-based modeling to simulate IPF progression and evaluate treatment efficacy by analyzing collagen accumulation and invasion under different treatment scenarios.