Shared and distinct mechanisms of fibrosis

JHW Distler, AH Györfi, M Ramanujam… - Nature Reviews …, 2019‏ - nature.com
Fibrosis is defined as an excessive deposition of connective tissue components and can
affect virtually every organ system, including the skin, lungs, liver and kidney. Fibrotic tissue …

Idiopathic pulmonary fibrosis

L Richeldi, HR Collard, MG Jones - The Lancet, 2017‏ - thelancet.com
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease.
Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is …

Single-cell RNA sequencing reveals profibrotic roles of distinct epithelial and mesenchymal lineages in pulmonary fibrosis

AC Habermann, AJ Gutierrez, LT Bui, SL Yahn… - Science …, 2020‏ - science.org
Pulmonary fibrosis (PF) is a form of chronic lung disease characterized by pathologic
epithelial remodeling and accumulation of extracellular matrix (ECM). To comprehensively …

Reference-based analysis of lung single-cell sequencing reveals a transitional profibrotic macrophage

D Aran, AP Looney, L Liu, E Wu, V Fong, A Hsu… - Nature …, 2019‏ - nature.com
Tissue fibrosis is a major cause of mortality that results from the deposition of matrix proteins
by an activated mesenchyme. Macrophages accumulate in fibrosis, but the role of specific …

SARS-CoV-2 infection produces chronic pulmonary epithelial and immune cell dysfunction with fibrosis in mice

KH Dinnon III, SR Leist, K Okuda, H Dang… - Science Translational …, 2022‏ - science.org
A subset of individuals who recover from coronavirus disease 2019 (COVID-19) develop
post-acute sequelae of severe acute respiratory syndrome coronavirus 2 (SARS-CoV …

Genetic variants associated with susceptibility to idiopathic pulmonary fibrosis in people of European ancestry: a genome-wide association study

RJ Allen, J Porte, R Braybrooke, C Flores… - The Lancet respiratory …, 2017‏ - thelancet.com
Background Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with
high mortality, uncertain cause, and few treatment options. Studies have identified a …

Mitochondrial NADP (H) generation is essential for proline biosynthesis

J Zhu, S Schwörer, M Berisa, YJ Kyung, KW Ryu, J Yi… - Science, 2021‏ - science.org
The coenzyme nicotinamide adenine dinucleotide phosphate (NADP+) and its reduced form
(NADPH) regulate reductive metabolism in a subcellularly compartmentalized manner …

Cilia dysfunction in lung disease

AE Tilley, MS Walters, R Shaykhiev… - Annual review of …, 2015‏ - annualreviews.org
A characteristic feature of the human airway epithelium is the presence of ciliated cells
bearing motile cilia, specialized cell surface projections containing axonemes composed of …

An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study

TM Maher, E Oballa, JK Simpson, J Porte… - The Lancet …, 2017‏ - thelancet.com
Background Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal disorder with a
variable disease trajectory. The aim of this study was to assess potential biomarkers to …

Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice

LA Hancock, CE Hennessy, GM Solomon… - Nature …, 2018‏ - nature.com
The gain-of-function MUC5B promoter variant rs35705950 is the dominant risk factor for
develo** idiopathic pulmonary fibrosis (IPF). Here we show in humans that MUC5B, a …