Altered ion transport by tracheal glands in cystic fibrosis

M Yamaya, WE Finkbeiner… - American Journal of …, 1991 - journals.physiology.org
Acini of tracheal glands from 12 humans without cystic fibrosis (CF) and from two CF patients
were isolated by enzymatic digestion. They were plated on flasks coated with human …

Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium

RD Coakley, BR Grubb, AM Paradiso… - Proceedings of the …, 2003 - National Acad Sciences
Cystic fibrosis (CF) transmembrane conductance regulator (CFTR)-dependent airway
epithelial bicarbonate transport is hypothesized to participate in airway surface liquid pH …

PBA increases CFTR expression but at high doses inhibits Cl secretion in Calu-3 airway epithelial cells

J Loffing, BD Moyer, D Reynolds… - American Journal of …, 1999 - journals.physiology.org
Sodium 4-phenylbutyrate (PBA), a short-chain fatty acid, has been approved to treat patients
with urea cycle enzyme deficiencies and is being evaluated in the management of sickle cell …

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 …

Inhibition of airway liquid secretion and its effect on the physical properties of airway mucus

L Trout, M King, W Feng, SK Inglis… - American Journal of …, 1998 - journals.physiology.org
The combination of both Cl− and HCO 3− secretion inhibitors causes an accumulation of
mucins within the submucosal gland ducts of acetylcholine (ACh)-treated bronchi [SK Inglis …

Chloride secretion by canine tracheal epithelium: II. The cellular electrical potential profile

MJ Welsh, PL Smith, RA Frizzell - The Journal of Membrane Biology, 1982 - Springer
We used intracellular microelectrode techniques to study the mechanisms responsible for Cl
secretion by canine tracheal epithelium. Tissues were treated with indomethacin (10− 6 m …

Airway surface liquid depth measured in ex vivo fragments of pig and human trachea: dependence on Na+ and Cl channel function

Y Song, W Namkung, DW Nielson… - … of Physiology-Lung …, 2009 - journals.physiology.org
The airway surface liquid (ASL) is the thin fluid layer lining the airways whose depth may be
reduced in cystic fibrosis. Prior measurements of ASL depth have been made in airway …

A small molecule CFTR inhibitor produces cystic fibrosis‐like submucosal gland fluid secretions in normal airways

JR Thiagarajah, Y Song, PM Haggie… - The FASEB …, 2004 - Wiley Online Library
Airway submucosal glands have been proposed as a primary site for initiating and
sustaining airway disease in cystic fibrosis (CF). However, it has been difficult to define the …

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 …

Activation of an apical Cl-conductance by Ca2+ ionophores in cystic fibrosis airway epithelia

NJ Willumsen, RC Boucher - American Journal of …, 1989 - journals.physiology.org
Cystic fibrosis (CF) airway epithelia express a defect in adenosine 3', 5'-cyclic
monophosphate (cAMP)-dependent regulation of apical membrane Cl-channels. Recent …