Animal models of mechanisms of SARS‐CoV‐2 infection and COVID‐19 pathology

SJ Cleary, SC Pitchford, RT Amison… - British journal of …, 2020 - Wiley Online Library
The coronavirus disease 2019 (COVID‐19) pandemic caused by SARS‐CoV‐2 infections
has led to a substantial unmet need for treatments, many of which will require testing in …

Cystic fibrosis genetics: from molecular understanding to clinical application

GR Cutting - Nature Reviews Genetics, 2015 - nature.com
The availability of the human genome sequence and tools for interrogating individual
genomes provide an unprecedented opportunity to apply genetics to medicine. Mendelian …

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

DT Montoro, AL Haber, M Biton, V Vinarsky, B Lin… - Nature, 2018 - nature.com
The airways of the lung are the primary sites of disease in asthma and cystic fibrosis. Here
we study the cellular composition and hierarchy of the mouse tracheal epithelium by single …

Molecular structure of the human CFTR ion channel

F Liu, Z Zhang, L Csanády, DC Gadsby, J Chen - Cell, 2017 - cell.com
The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding
cassette (ABC) transporter that uniquely functions as an ion channel. Here, we present a 3.9 …

Origins of cystic fibrosis lung disease

DA Stoltz, DK Meyerholz, MJ Welsh - New England Journal of …, 2015 - Mass Medical Soc
Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid …

[HTML][HTML] Transgenic ferret models define pulmonary ionocyte diversity and function

F Yuan, GN Gasser, E Lemire, DT Montoro… - Nature, 2023 - nature.com
Speciation leads to adaptive changes in organ cellular physiology and creates challenges
for studying rare cell-type functions that diverge between humans and mice. Rare cystic …

Dual SMAD signaling inhibition enables long-term expansion of diverse epithelial basal cells

H Mou, V Vinarsky, PR Tata, K Brazauskas, SH Choi… - Cell stem cell, 2016 - cell.com
Functional modeling of many adult epithelia is limited by the difficulty in maintaining relevant
stem cell populations in culture. Here, we show that dual inhibition of SMAD signaling …

An official American Thoracic Society workshop report: use of animal models for the preclinical assessment of potential therapies for pulmonary fibrosis

RG Jenkins, BB Moore, RC Chambers… - American journal of …, 2017 - atsjournals.org
Numerous compounds have shown efficacy in limiting development of pulmonary fibrosis
using animal models, yet few of these compounds have replicated these beneficial effects in …

The innate immune function of airway epithelial cells in inflammatory lung disease

PS Hiemstra, PB McCray Jr… - European respiratory …, 2015 - publications.ersnet.org
The airway epithelium is now considered to be central to the orchestration of pulmonary
inflammatory and immune responses, and is also key to tissue remodelling. It acts as the first …

Functional gene correction for cystic fibrosis in lung epithelial cells generated from patient iPSCs

AL Firth, T Menon, GS Parker, SJ Qualls, BM Lewis… - Cell reports, 2015 - cell.com
Lung disease is a major cause of death in the United States, with current therapeutic
approaches serving only to manage symptoms. The most common chronic and life …