Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry

A Zsembery, JA Fortenberry, L Liang, Z Bebok… - Journal of Biological …, 2004 - ASBMB
Cystic fibrosis (CF) is caused by defective cyclic AMP-dependent cystic fibrosis
transmembrane conductance regulator Cl-channels. Thus, CF epithelia fail to transport Cl …

RNA interference targeted to multiple P2X receptor subtypes attenuates zinc-induced calcium entry

L Liang, A Zsembery… - American Journal of …, 2005 - journals.physiology.org
A postulated therapeutic avenue in cystic fibrosis (CF) is activation of Ca2+-dependent Cl−
channels via stimulation of Ca2+ entry from extracellular solutions independent of CFTR …

Calcium-stimulated Clsecretion in Calu-3 human airway cells requires CFTR

S Moon, M Singh, ME Krouse… - American Journal of …, 1997 - journals.physiology.org
Human airway serous cells secrete antibiotic-rich fluid, but, in cystic fibrosis (CF), Cl−-
dependent fluid secretion is impaired by defects in CF transmembrane conductance …

Ca2+-Activated Cl Channels: A Newly Emerging Anion Transport Family

CM Fuller, DJ Benos - Physiology, 2000 - journals.physiology.org
A new family of Cl–channels widely expressed in epithelia has been identified. These
proteins are associated with Ca2+-sensitive conductive Cl–transport when heterologously …

[HTML][HTML] F508del-CFTR increases intracellular Ca2+ signaling that causes enhanced calcium-dependent Cl− conductance in cystic fibrosis

JR Martins, P Kongsuphol, E Sammels… - … et Biophysica Acta (BBA …, 2011 - Elsevier
In many cells, increase in intracellular calcium ([Ca2+] i) activates a Ca2+-dependent
chloride (Cl−) conductance (CaCC). CaCC is enhanced in cystic fibrosis (CF) epithelial cells …

Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR

F Antigny, C Norez, F Becq, C Vandebrouck - Cell calcium, 2008 - Elsevier
Retention of F508del-CFTR proteins in the endoplasmic reticulum (ER) is dependent upon
chaperone proteins, many of which require Ca2+ for optimal activity. Here, we show in …

Chloride secretory response to extracellular ATP in human normal and cystic fibrosis nasal epithelia

LL Clarke, RC Boucher - American Journal of Physiology …, 1992 - journals.physiology.org
Chloride secretion across cystic fibrosis (CF) airway epithelia is effectively regulated by
pathways associated with intracellular Ca2+ metabolism, but not by mechanisms dependent …

Vitamin C controls the cystic fibrosis transmembrane conductance regulator chloride channel

H Fischer, C Schwarzer, B Illek - Proceedings of the …, 2004 - National Acad Sciences
Vitamin C (l-ascorbate) is present in the respiratory lining fluid of human lungs, and local
deficits occur during oxidative stress. Here we report a unique function of vitamin C on the …

CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

EM Schwiebert, ME Egan, TH Hwang, SB Fulmer… - Cell, 1995 - cell.com
The cystic fibrosis transmembrane conductance regulator (CFTR) functions to regulate both
CI− and Na+ conductive pathways; however, the cellular mechanisms whereby CFTR acts …

Human cystic fibrosis airway epithelia have reduced Cl conductance but not increased Na+ conductance

OA Itani, JH Chen, PH Karp, S Ernst… - Proceedings of the …, 2011 - National Acad Sciences
Loss of cystic fibrosis transmembrane conductance regulator (CFTR) anion channel function
causes cystic fibrosis (CF) lung disease. CFTR is expressed in airway epithelia, but how CF …