Role of CFTR in epithelial physiology

V Saint-Criq, MA Gray - Cellular and Molecular Life Sciences, 2017 - Springer
Salt and fluid absorption and secretion are two processes that are fundamental to epithelial
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …

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 …

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

DT Montoro, AL Haber, M Biton, V Vinarsky, B Lin… - Nature, 2018 - nature.com
The airways of the lung are the primary sites of disease in asthma and cystic fibrosis. Here
we study the cellular composition and hierarchy of the mouse tracheal epithelium by single …

Well-differentiated human airway epithelial cell cultures

ML Fulcher, S Gabriel, KA Burns, JR Yankaskas… - Human cell culture …, 2005 - Springer
The airway epithelium occupies a critical environmental interface, protecting the host from a
wide variety of inhaled insults, including chemical and particulate pollutants and pathogens …

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

DM Cholon, NL Quinney, ML Fulcher… - Science translational …, 2014 - science.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR). Newly developed “correctors” such as lumacaftor (VX-809) that improve CFTR …

Calcium-activated chloride channels

C Hartzell, I Putzier, J Arreola - Annu. Rev. Physiol., 2005 - annualreviews.org
▪ Abstract Calcium-activated chloride channels (CaCCs) play important roles in cellular
physiology, including epithelial secretion of electrolytes and water, sensory transduction …

State of the art: why do the lungs of patients with cystic fibrosis become infected and why can't they clear the infection?

JF Chmiel, PB Davis - Respiratory research, 2003 - Springer
Cystic Fibrosis (CF) lung disease, which is characterized by airway obstruction, chronic
bacterial infection, and an excessive inflammatory response, is responsible for most of the …

Voltage-and calcium-dependent gating of TMEM16A/Ano1 chloride channels are physically coupled by the first intracellular loop

Q Xiao, K Yu, P Perez-Cornejo, Y Cui… - Proceedings of the …, 2011 - National Acad Sciences
Ca2+-activated Cl− channels (CaCCs) are exceptionally well adapted to subserve diverse
physiological roles, from epithelial fluid transport to sensory transduction, because their …

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 …

Small-molecule screen identifies inhibitors of a human intestinal calcium-activated chloride channel

R De La Fuente, W Namkung, A Mills… - Molecular pharmacology, 2008 - ASPET
Calcium-activated chloride channels (CaCCs) are widely expressed in mammalian tissues,
including intestinal epithelia, where they facilitate fluid secretion. Potent, selective CaCC …