Characterization of the oligomeric structure of the Ca2+-activated Cl− channel Ano1/TMEM16A
JT Sheridan, EN Worthington, K Yu, SE Gabriel… - Journal of Biological …, 2011 - ASBMB
Members of the Anoctamin (Ano)/TMEM16A family have recently been identified as
essential subunits of the Ca 2+-activated chloride channel (CaCC). For example, Ano1 is …
essential subunits of the Ca 2+-activated chloride channel (CaCC). For example, Ano1 is …
Structure and function of CLCA proteins
ME Loewen, GW Forsyth - Physiological reviews, 2005 - journals.physiology.org
CLCA proteins were discovered in bovine trachea and named for a calcium-dependent
chloride conductance found in trachea and in other secretory epithelial tissues. At least four …
chloride conductance found in trachea and in other secretory epithelial tissues. At least four …
[HTML][HTML] Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation
AT Trimble, SH Donaldson - Journal of Cystic Fibrosis, 2018 - Elsevier
Ivacaftor use can lead to dramatic health improvements in cystic fibrosis (CF) patients with
gating mutations. Here, we report five instances of dramatic clinical decline following …
gating mutations. Here, we report five instances of dramatic clinical decline following …
Changes in ion transport in inflammatory disease
M Eisenhut - Journal of inflammation, 2006 - Springer
Ion transport is essential for maintenance of transmembranous and transcellular electric
potential, fluid transport and cellular volume. Disturbance of ion transport has been …
potential, fluid transport and cellular volume. Disturbance of ion transport has been …
17β-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia
RD Coakley, H Sun, LA Clunes… - The Journal of …, 2008 - Am Soc Clin Investig
Normal airways homeostatically regulate the volume of airway surface liquid (ASL) through
both cAMP-and Ca2+-dependent regulation of ion and water transport. In cystic fibrosis (CF) …
both cAMP-and Ca2+-dependent regulation of ion and water transport. In cystic fibrosis (CF) …
The ΔF508 mutation results in loss of CFTR function and mature protein in native human colon
M Mall, SM Kreda, A Mengos, TJ Jensen, S Hirtz… - Gastroenterology, 2004 - Elsevier
Background & Aims: Deletion of the codon for phenylalanine at position 508 (ΔF508) is the
most frequent disease-causing mutation in the cystic fibrosis transmembrane conductance …
most frequent disease-causing mutation in the cystic fibrosis transmembrane conductance …
The therapeutic potential of CFTR modulators for COPD and other airway diseases
GM Solomon, L Fu, SM Rowe, JF Collawn - Current opinion in …, 2017 - Elsevier
Highlights•Common airway diseases including COPD, asthma and non-CF bronchiectasis
(NCFBE) share the common pathophysiologic feature of mucus obstruction.•Acquired CFTR …
(NCFBE) share the common pathophysiologic feature of mucus obstruction.•Acquired CFTR …
The TMEM16A chloride channel as an alternative therapeutic target in cystic fibrosis
E Sondo, E Caci, LJV Galietta - The international journal of biochemistry & …, 2014 - Elsevier
Cystic fibrosis (CF), a multiorgan genetic disease, is caused by loss of function of CFTR, a
cAMP-regulated anion channel. In CF airway epithelia, defective Cl− and bicarbonate …
cAMP-regulated anion channel. In CF airway epithelia, defective Cl− and bicarbonate …
Liquid movement across the surface epithelium of large airways
LA Chambers, BM Rollins, R Tarran - Respiratory physiology & …, 2007 - Elsevier
The cystic fibrosis transmembrane conductance regulator CFTR gene is found on
chromosome 7 [Kerem, B., Rommens, JM, Buchanan, JA, Markiewicz, D., Cox, TK …
chromosome 7 [Kerem, B., Rommens, JM, Buchanan, JA, Markiewicz, D., Cox, TK …
[HTML][HTML] CFTR-regulated chloride transport at the ocular surface in living mice measured by potential differences
MH Levin, AS Verkman - Investigative ophthalmology & visual …, 2005 - arvojournals.org
purpose. To define the role of the cystic fibrosis transmembrane conductance regulator
(CFTR) in Cl− secretion at the mouse ocular surface in vivo. methods. Open-circuit potential …
(CFTR) in Cl− secretion at the mouse ocular surface in vivo. methods. Open-circuit potential …