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 …

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 …

Animal models in the pathophysiology of cystic fibrosis

A Semaniakou, RP Croll, V Chappe - Frontiers in pharmacology, 2019 - frontiersin.org
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 …

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 …

Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl–secretory channel in mouse airways

JR Rock, WK O'Neal, SE Gabriel, SH Randell… - Journal of Biological …, 2009 - ASBMB
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 …

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 …

Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy

B Lubamba, B Dhooghe, S Noel, T Leal - Clinical biochemistry, 2012 - Elsevier
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 …

Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children

DK Meyerholz, DA Stoltz, E Namati… - American journal of …, 2010 - atsjournals.org
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 …

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 …

What role does CFTR play in development, differentiation, regeneration and cancer?

MD Amaral, MC Quaresma, I Pankonien - International journal of …, 2020 - mdpi.com
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 …