Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

Cystic fibrosis and Pseudomonas aeruginosa: the host-microbe interface

S Malhotra, D Hayes Jr, DJ Wozniak - Clinical microbiology …, 2019 - Am Soc Microbiol
In human pathophysiology, the clash between microbial infection and host immunity
contributes to multiple diseases. Cystic fibrosis (CF) is a classical example of this …

Cilia and mucociliary clearance

XM Bustamante-Marin… - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Mucociliary clearance (MCC) is the primary innate defense mechanism of the lung. The
functional components are the protective mucous layer, the airway surface liquid layer, and …

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 …

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 …

Inflammation in cystic fibrosis: An update

EA Roesch, DP Nichols, JF Chmiel - Pediatric pulmonology, 2018 - Wiley Online Library
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease
progression making it an active area of research and important therapeutic target. In this …

Muc5b is required for airway defence

MG Roy, A Livraghi-Butrico, AA Fletcher, MM McElwee… - Nature, 2014 - nature.com
Respiratory surfaces are exposed to billions of particulates and pathogens daily. A
protective mucus barrier traps and eliminates them through mucociliary clearance (MCC) …

Harnessing inhaled nanoparticles to overcome the pulmonary barrier for respiratory disease therapy

Z Jin, Q Gao, K Wu, J Ouyang, W Guo… - Advanced Drug Delivery …, 2023 - Elsevier
The lack of effective treatments for pulmonary diseases presents a significant global health
burden, primarily due to the challenges posed by the pulmonary barrier that hinders drug …

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

AA Pezzulo, XX Tang, MJ Hoegger, MH Abou Alaiwa… - Nature, 2012 - nature.com
Cystic fibrosis (CF) is a life-shortening disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …

Structure and function of TMEM16 proteins (anoctamins)

N Pedemonte, LJV Galietta - Physiological reviews, 2014 - journals.physiology.org
TMEM16 proteins, also known as anoctamins, are involved in a variety of functions that
include ion transport, phospholipid scrambling, and regulation of other membrane proteins …