Pathogenic mechanisms underlying idiopathic pulmonary fibrosis

BJ Moss, SW Ryter, IO Rosas - Annual Review of Pathology …, 2022 - annualreviews.org
The pathogenesis of idiopathic pulmonary fibrosis (IPF) involves a complex interplay of cell
types and signaling pathways. Recurrent alveolar epithelial cell (AEC) injury may occur in …

Idiopathic pulmonary fibrosis: an update on pathogenesis

Q Mei, Z Liu, H Zuo, Z Yang, J Qu - Frontiers in pharmacology, 2022 - frontiersin.org
Idiopathic pulmonary fibrosis (IPF) is a progressive, lethal fibrotic lung disease that occurs
primarily in middle-aged and elderly adults. It is a major cause of morbidity and mortality …

Idiopathic pulmonary fibrosis: Disease mechanisms and drug development

P Spagnolo, JA Kropski, MG Jones, JS Lee… - Pharmacology & …, 2021 - Elsevier
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease of unknown cause
characterized by relentless scarring of the lung parenchyma leading to reduced quality of life …

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 …

The landscape of GWAS validation; systematic review identifying 309 validated non-coding variants across 130 human diseases

AJ Alsheikh, S Wollenhaupt, EA King, J Reeb… - BMC medical …, 2022 - Springer
Background The remarkable growth of genome-wide association studies (GWAS) has
created a critical need to experimentally validate the disease-associated variants, 90% of …

Prevalence and mechanisms of mucus accumulation in COVID-19 lung disease

T Kato, T Asakura, CE Edwards, H Dang… - American journal of …, 2022 - atsjournals.org
Rationale: The incidence and sites of mucus accumulation and molecular regulation of
mucin gene expression in coronavirus (COVID-19) lung disease have not been reported …

[HTML][HTML] IL-1β dominates the promucin secretory cytokine profile in cystic fibrosis

G Chen, L Sun, T Kato, K Okuda, MB Martino… - The Journal of clinical …, 2019 - jci.org
Cystic fibrosis (CF) lung disease is characterized by early and persistent mucus
accumulation and neutrophilic inflammation in the distal airways. Identification of the factors …

Emerging roles of airway epithelial cells in idiopathic pulmonary fibrosis

A Chakraborty, M Mastalerz, M Ansari, HB Schiller… - Cells, 2022 - mdpi.com
Idiopathic pulmonary fibrosis (IPF) is a fatal disease with incompletely understood aetiology
and limited treatment options. Traditionally, IPF was believed to be mainly caused by …

[HTML][HTML] Contributions of alveolar epithelial cell quality control to pulmonary fibrosis

J Katzen, MF Beers - The Journal of clinical investigation, 2020 - jci.org
Epithelial cell dysfunction has emerged as a central component of the pathophysiology of
diffuse parenchymal diseases including idiopathic pulmonary fibrosis (IPF). Alveolar type 2 …

Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration

CB Morrison, KM Shaffer, KC Araba… - European …, 2022 - publications.ersnet.org
Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis …