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 …
undermine many host defense systems by inhibiting the function of airway-surface liquid …
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 …
Animal models in the pathophysiology of cystic fibrosis
Our understanding of the multiorgan pathology of cystic fibrosis (CF) has improved
impressively during the last decades, but we still lack a full comprehension of the disease …
impressively during the last decades, but we still lack a full comprehension of the disease …
Mouse models of cystic fibrosis: phenotypic analysis and research applications
M Wilke, RM Buijs-Offerman, J Aarbiou… - Journal of Cystic …, 2011 - Elsevier
Genetically modified mice have been studied for more than fifteen years as models of cystic
fibrosis (CF). The large amount of experimental data generated illuminates the complex …
fibrosis (CF). The large amount of experimental data generated illuminates the complex …
Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl–secretory channel in mouse airways
For almost two decades, it has been postulated that calcium-activated Cl–channels (CaCCs)
play a role in airway epithelial Cl–secretion, but until recently, the molecular identity of the …
play a role in airway epithelial Cl–secretion, but until recently, the molecular identity of the …
The ΔF508 Mutation Causes CFTR Misprocessing and Cystic Fibrosis–Like Disease in Pigs
LS Ostedgaard, DK Meyerholz, JH Chen… - Science translational …, 2011 - science.org
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene
encoding the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel …
encoding the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel …
Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy
Cystic fibrosis is the most common life-threatening recessively inherited disease in
Caucasians. Due to early provision of care in specialized reference centers and more …
Caucasians. Due to early provision of care in specialized reference centers and more …
Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children
Rationale: Although airway abnormalities are common in patients with cystic fibrosis (CF), it
is unknown whether they are all secondary to postnatal infection and inflammation, which …
is unknown whether they are all secondary to postnatal infection and inflammation, which …
Inflammatory diseases and growth: effects on the GH–IGF axis and on growth plate
F Cirillo, P Lazzeroni, C Sartori, ME Street - International Journal of …, 2017 - mdpi.com
This review briefly describes the most common chronic inflammatory diseases in childhood,
such as cystic fibrosis (CF), inflammatory bowel diseases (IBDs), juvenile idiopathic arthritis …
such as cystic fibrosis (CF), inflammatory bowel diseases (IBDs), juvenile idiopathic arthritis …
What role does CFTR play in development, differentiation, regeneration and cancer?
One of the key features associated with the substantial increase in life expectancy for
individuals with CF is an elevated predisposition to cancer, firmly established by recent …
individuals with CF is an elevated predisposition to cancer, firmly established by recent …