Results 11 to 20 of about 31,972 (221)
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
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CFTR and TNR-CFTR expression and function in the kidney [PDF]
Biophysical Reviews, 2014 The cystic fibrosis transmembrane conductance regulator (CFTR) is abundantly expressed in the kidney. CFTR mRNA is detected in all nephron segments of rats and humans and its expression is higher in the renal cortex and outer medulla than in the inner medulla.
Jackson, Souza-Menezes +2 more
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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
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Development of CFTR structure [PDF]
Frontiers in Pharmacology, 2012 Cystic fibrosis is a lethal genetic disease caused by lack of functional cystic fibrosis transmembrane conductance regulator (CFTR) proteins at the apical surface of secretory epithelia. CFTR is a multidomain protein, containing five domains, and its functional structure is attained in a hierarchical folding process.
Patrick, Anna E., Thomas, Philip J.
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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
FUNCIÓN PULMONAR EN FIBROSIS QUÍSTICA
Neumología Pediátrica, 2022 La fibrosis quística (FQ) es una enfermedad hereditaria autosómica recesiva, causada por la mutación del gen que codifica la proteína CFTR (cystic fibrosis transmembrane conductance regulator), afecta varios órganos, pero la enfermedad pulmonar es la ...
Hortensia Barrientos
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Journal of the Belgian Society of Radiology, 2022 Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy effects on respiratory function, pulmonary exacerbations and quality of life have been well documented. However, CFTR modulator therapy effects on sinus disease have
Corrado Tagliati +10 more
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Current Pharmaceutical Design, 2013 The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a cAMP-regulated Cl- channel whose major function is to facilitate epithelial fluid secretion. Loss-of-function mutations in CFTR cause the genetic disease cystic fibrosis. CFTR is required for transepithelial fluid transport in certain secretory diarrheas, such as cholera, and ...
Verkman, Alan S +4 more
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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
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Biology Open, 2020 Cystic fibrosis (CF) is a genetic disease characterized by progressive lung and chronic digestive manifestations. We have shown that therapeutic doses of vardenafil, a phosphodiesterase type 5 (PDE5) inhibitor, corrects CF Transmembrane conductance ...
Barbara Dhooghe +7 more
doaj +1 more source