Physiology and pathophysiology of human airway mucus
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 …
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
In human pathophysiology, the clash between microbial infection and host immunity
contributes to multiple diseases. Cystic fibrosis (CF) is a classical example of this …
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 …
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
Rationale: Identification of the specific cell types expressing CFTR (cystic fibrosis [CF]
transmembrane conductance regulator) is required for precision medicine therapies for 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 …
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 …
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) …
protective mucus barrier traps and eliminates them through mucociliary clearance (MCC) …
Harnessing inhaled nanoparticles to overcome the pulmonary barrier for respiratory disease therapy
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 …
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 …
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 …
include ion transport, phospholipid scrambling, and regulation of other membrane proteins …