Results 11 to 20 of about 81,604 (317)
The Journal of General Physiology, 2001 Some studies of CFTR imply that channel activation can be explained by an increase in open probability (Po), whereas others suggest that activation involves an increase in the number of CFTR channels (N) in the plasma membrane. Using two-electrode voltage clamp, we tested for changes in N associated with activation of CFTR in Xenopus oocytes using a ...
Xuehong Liu +3 more
openaire +3 more sources
CFTR mutations altering CFTR fragmentation [PDF]
Biochemical Journal, 2012 Most CF (cystic fibrosis) results from deletion of a phenylalanine (F508) in the CFTR {CF transmembrane-conductance regulator; ABCC7 [ABC (ATP-binding cassette) sub-family C member 7]} which causes ER (endoplasmic reticulum) degradation of the mutant. Using stably CFTR-expressing BHK (baby-hamster kidney) cell lines we demonstrated that wild-type CTFR ...
TOSONI, KENDRA +10 more
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Distinct Roles of SLC26A3 and CFTR in Surface pH Regulation and Bicarbonate Secretion in Human Intestinal Epithelium. [PDF]
Acta Physiol (Oxf)ABSTRACT Background and Aims Colonic bicarbonate secretion is mediated by the chloride/bicarbonate exchanger SLC26A3 and the cystic fibrosis transmembrane conductance regulator (CFTR). Dysfunction of either causes luminal acidosis, altered mucus properties, and inflammation.
Amiri M, Salari A, Seidler U.
europepmc +2 more sources
Journal of Cystic Fibrosis, 2018 Structural studies of the cystic fibrosis transmembrane conductance regulator (CFTR) protein are critical to understand molecular mechanisms involved in gating of the apical anion channel as well as the way in which the gating is regulated, especially by the regulatory region (R region).
Isabelle, Callebaut +2 more
openaire +2 more sources
Disease Models & Mechanisms, 2021 Infection by multidrug-resistant Mycobacterium abscessus is increasingly prevalent in cystic fibrosis (CF) patients, leaving clinicians with few therapeutic options.
Matt D. Johansen +6 more
doaj +1 more source
Mechanisms of endothelial cell dysfunction in cystic fibrosis [PDF]
, 2017 Although cystic fibrosis (CF) patients exhibit signs of endothelial perturbation, the functions of the cystic fibrosis conductance regulator (CFTR) in vascular endothelial cells (EC) are poorly defined.
Anile, Marco +22 more
core +1 more source
Physiological parameters of Brazilian silverside, Atherinella brasiliensis, embryos exposed to different salinities [PDF]
Neotropical IchthyologyInformation regarding organism changes due to the variation of abiotic factors such as salinity are essential in both ecotoxicological and environmental monitoring studies. For this reason, the Brazilian silverside (Atherinella brasiliensis) embryos were
Carolina Brioschi Delpupo +5 more
doaj +1 more source
New era of cystic fibrosis: full mutational analysis and personalized therapy [PDF]
, 2017 Despite its apparently simple genetics, cystic fibrosis (CF) is a rather complex genetic disease. A lot of variability in the steps of the path from the cystic fibrosis transmembrane conductance regulator (CFTR ) gene to the clinical manifestations ...
Lucarelli, Marco
core +1 more source
CFTR Modulator Therapy for Rare CFTR Mutants
Journal of Respiration, 2022 Cystic fibrosis (CF), the most common genetic disease among the Caucasian population, is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR), a chloride epithelial channel whose dysfunction results in severe airway obstruction and inflammation, eventually leading to respiratory failure.
Marco Mergiotti +3 more
openaire +2 more sources
Biology Open, 2021 Claudin (Cldn)-10 tight junction (TJ) proteins are hypothesized to form the paracellular Na+ secretion pathway of hyposmoregulating mummichog (Fundulus heteroclitus) branchial epithelia.
Chun Chih Chen +4 more
doaj +1 more source