Pulmonary Fibrosis Ferret Model Demonstrates Sustained Fibrosis, Restrictive Physiology, and Aberrant Repair

JE Peabody Lever, Q Li, N Pavelkova, SS Hussain… - bioRxiv, 2024 - biorxiv.org
Rationale: The role of MUC5B mucin expression in IPF pathogenesis is unknown.
Bleomycin-exposed rodent models do not exhibit sustained fibrosis or airway remodeling …

Epithelial endoplasmic reticulum stress enhances the risk of Muc5b-associated lung fibrosis

E Dobrinskikh, CE Hennessy, JS Kurche… - American Journal of …, 2023 - atsjournals.org
The gain-of-function minor allele of the MUC5B (mucin 5B, oligomeric mucus/gel-forming)
promoter (rs35705950) is the strongest risk factor for idiopathic pulmonary fibrosis (IPF), a …

Persistent, progressive pulmonary fibrosis and epithelial remodeling in mice

EF Redente, BP Black, DS Backos… - American journal of …, 2021 - atsjournals.org
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with
underlying mechanisms that have been primarily investigated in mice after intratracheal …

Genes, other than Muc5b, play a role in bleomycin-induced lung fibrosis

E Dobrinskikh, AM Estrella… - … of Physiology-Lung …, 2021 - journals.physiology.org
Idiopathic pulmonary fibrosis (IPF) is an incurable genetic disease that affects 5 million
people worldwide. The gain-of-function MUC5B promoter variant rs35705950 is the …

Genetic Locus Associated With Bleomycin Induced Lung Fibrosis in Mice

Y Wang, C Hennessy, K Hatakka… - A71. CUTTING EDGE …, 2024 - atsjournals.org
Idiopathic pulmonary fibrosis (IPF) is a complex genetic disease involving both rare and
common genetic variants in its pathogenesis. One prominent common gain-of-function …

In vivo MRI and PET imaging in a translational ILD mouse model expressing non-resolving fibrosis and bronchiectasis-like pathology after repeated systemic …

I Mahmutovic Persson, N Fransén Petterson… - Frontiers in …, 2024 - frontiersin.org
Drug-induced interstitial lung disease (ILD) is crucial to detect early to achieve the best
treatment outcome. Optimally, non-invasive imaging biomarkers can be used for early …

Transcriptomic and proteomic changes driving pulmonary fibrosis resolution in young and old mice

J Weckerle, CH Mayr, K Fundel-Clemens… - American Journal of …, 2023 - atsjournals.org
Bleomycin-induced pulmonary fibrosis in mice mimics major hallmarks of idiopathic
pulmonary fibrosis. Yet in this model, it spontaneously resolves over time. We studied …

Muc5b Overexpression and Aging Results in Lung Fibrosis in Mice

C Hennessy, E Dobrinskikh, AC Russell… - B28. LUNGS …, 2024 - atsjournals.org
RATIONALE: We have previously found that Muc5b overexpression causes mucociliary
dysfunction and enhances lung fibrosis in SFTPC-Muc5bTg mice exposed to bleomycin …

[HTML][HTML] Pulmonary fibrosis distal airway epithelia are dynamically and structurally dysfunctional

IT Stancil, JE Michalski, D Davis-Hall, HW Chu… - Nature …, 2021 - nature.com
The airway epithelium serves as the interface between the host and external environment. In
many chronic lung diseases, the airway is the site of substantial remodeling after injury …

[HTML][HTML] Insights into Disease Progression of Translational Preclinical Rat Model of Interstitial Pulmonary Fibrosis through Endpoint Analysis

AH Kadam, JE Schnitzer - Cells, 2024 - mdpi.com
Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease characterized by
the relentless deposition of extracellular matrix (ECM), causing lung distortions and …