The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches
SY Graeber, MA Mall - The Lancet, 2023 - thelancet.com
With the 2019 breakthrough in the development of highly effective modulator therapy
providing unprecedented clinical benefits for over 90% of patients with cystic fibrosis who …
providing unprecedented clinical benefits for over 90% of patients with cystic fibrosis who …
Harnessing three-dimensional (3D) cell culture models for pulmonary infections: State of the art and future directions
Pulmonary infections have been a leading etiology of morbidity and mortality worldwide.
Upper and lower respiratory tract infections have multifactorial causes, which include …
Upper and lower respiratory tract infections have multifactorial causes, which include …
[HTML][HTML] Altering the viscoelastic properties of mucus-grown Pseudomonas aeruginosa biofilms affects antibiotic susceptibility
The viscoelastic properties of biofilms are correlated with their susceptibility to mechanical
and chemical stress, and the airway environment in muco-obstructive pulmonary diseases …
and chemical stress, and the airway environment in muco-obstructive pulmonary diseases …
Emerging imaging modalities for functional assessment of donor lungs ex vivo
The severe shortage of functional donor lungs that can be offered to recipients has been a
major challenge in lung transplantation. Innovative ex vivo lung perfusion (EVLP) and tissue …
major challenge in lung transplantation. Innovative ex vivo lung perfusion (EVLP) and tissue …
Mucociliary clearance is impaired in small airways of cystic fibrosis pigs
CG Stewart, BM Hilkin, ND Gansemer… - … of Physiology-Lung …, 2024 - journals.physiology.org
Cystic fibrosis (CF) is a genetic disorder characterized by recurrent airway infections,
inflammation, impaired mucociliary clearance, and progressive decline in lung function. The …
inflammation, impaired mucociliary clearance, and progressive decline in lung function. The …
The pH-sensitive translocation of V-ATPase in the small airway of cystic fibrosis pigs
R Villacreses, IM Thornell, X Li… - … of Physiology-Lung …, 2024 - journals.physiology.org
In contrast to pig large airways, the pH of airway surface liquid (ASL) in pig small airways is
regulated by CFTR-mediated HCO–3 secretion and the vacuolar-type H+ ATPase (V …
regulated by CFTR-mediated HCO–3 secretion and the vacuolar-type H+ ATPase (V …
Tromethamine improves mucociliary clearance in cystic fibrosis pigs
JJ Ash, BM Hilkin, ND Gansemer… - Physiological …, 2022 - Wiley Online Library
In cystic fibrosis (CF), the loss of cystic fibrosis transmembrane conductance regulator
(CFTR) mediated Cl− and HCO3− secretion across the epithelium acidifies the airway …
(CFTR) mediated Cl− and HCO3− secretion across the epithelium acidifies the airway …
A mechano-diffusion characterization platform for probing strain-programmable nanoparticle diffusion in hydrogels
Nanoparticle diffusion is a fundamental process that ubiquitously exists in life science and
engineering technology. Recent studies demonstrate the potential of harnessing mechanical …
engineering technology. Recent studies demonstrate the potential of harnessing mechanical …
Magnetic resonance brain volumetry biomarkers of CLN2 Batten disease identified with miniswine model
Late-infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease (Batten disease) is a
rare pediatric disease, with symptom development leading to clinical diagnosis. Early …
rare pediatric disease, with symptom development leading to clinical diagnosis. Early …
Inferring patterns of recombination and divergence with ancient and modern treponemal genomes
The treponemal diseases yaws, bejel and syphilis, all caused by subspecies of the
bacterium Treponema pallidum, are re-emerging worldwide, yet their origins and spread …
bacterium Treponema pallidum, are re-emerging worldwide, yet their origins and spread …