CGRP induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice

W Xie, JT Fisher, TJ Lynch, M Luo… - The Journal of …, 2011 - Am Soc Clin Investig
In cystic fibrosis (CF), a lack of functional CF transmembrane conductance regulator (CFTR)
chloride channels causes defective secretion by submucosal glands (SMGs), leading to …

Murine submucosal glands are clonally derived and show a cystic fibrosis gene–dependent distribution pattern

DW Borthwick, JD West, MA Keighren… - American journal of …, 1999 - atsjournals.org
Submucosal glands (SMGs) are the major site of expression of the cystic fibrosis (CF)
transmembrane conductance regulator gene (CFTR) in the human lung. As such, SMGs may …

Secretory cells dominate airway CFTR expression and function in human airway superficial epithelia

K Okuda, H Dang, Y Kobayashi, G Carraro… - American journal of …, 2021 - atsjournals.org
Rationale: Identification of the specific cell types expressing CFTR (cystic fibrosis [CF]
transmembrane conductance regulator) is required for precision medicine therapies for CF …

Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased β1-integrin signaling and delayed wound repair

Y Itokazu, RE Pagano, AS Schroeder… - … of Physiology-Cell …, 2014 - journals.physiology.org
Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function reduces
chloride secretion and increases sodium uptake, but it is not clear why CFTR mutation also …

Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice

JP Ianowski, JY Choi, JJ Wine… - The Journal of …, 2007 - Wiley Online Library
Submucosal glands line the cartilaginous airways and produce most of the antimicrobial
mucus that keeps the airways sterile. The glands are defective in cystic fibrosis (CF), but how …

Challenges facing airway epithelial cell-based therapy for cystic fibrosis

A Berical, RE Lee, SH Randell… - Frontiers in pharmacology, 2019 - frontiersin.org
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause
the life-limiting hereditary disease, cystic fibrosis (CF). Decreased or absent functional CFTR …

Why mouse airway submucosal gland serous cells do not secrete fluid in response to cAMP stimulation

RJ Lee, JK Foskett - Journal of Biological Chemistry, 2012 - ASBMB
Airway submucosal glands are important sites of cystic fibrosis transmembrane conductance
regulator (CFTR) chloride (Cl−) channel expression and fluid secretion in the airway …

A single-cell atlas of large and small airways at birth in a porcine model of cystic fibrosis

AL Thurman, X Li, R Villacreses, W Yu… - American journal of …, 2022 - atsjournals.org
Lack of CFTR (cystic fibrosis transmembrane conductance regulator) affects the
transcriptome, composition, and function of large and small airway epithelia in people with …

The role of specialized pro-resolving mediators in cystic fibrosis airways disease

M Briottet, M Shum, V Urbach - Frontiers in Pharmacology, 2020 - frontiersin.org
Cystic Fibrosis (CF) is a recessive genetic disease due to mutations of the Cystic Fibrosis
Transmembrane Conductance Regulator (CFTR) gene encoding the CFTR chloride …

Characterization of novel airway submucosal gland cell models for cystic fibrosis

A da Paula, A Ramalho, C Farinha, J Cheung… - Cellular Physiology and …, 2005 - karger.com
Cultured airway epithelial cells are widely used in cystic fibrosis (CF) research as in vitro
models that mimic the in vivo manifestations of the disease and help to define a specific …