Results 81 to 90 of about 5,299 (208)

Impaired Renal Base Excretion in Secretin Receptor Knock‐Out Mice During Prolonged Base‐Loading

Acta Physiologica, Volume 242, Issue 9, September 2026.
ABSTRACT Aim Secretin was recently found to play a pivotal role in the renal adaptation to acute base excess. Here, secretin increases pendrin‐dependent HCO3− secretion from the beta‐intercalated cells in the cortical collecting ducts. Whether secretin and its receptor play a role during prolonged base‐loading remains unknown.
Tobias Jensen, Jesper Frank Andersen, Laura Woidemann Trans, Ida Marie Modvig, Helga Vitzthum, Jens Juul Holst, Bolette Hartman, Samuel L. Svendsen, Mads Vaarby Sørensen, Jens Leipziger, Peder Berg   +10 more
wiley   +1 more source

A Quarter Century of EHD Protein Research: From Endosomal Recycling to Ciliopathies

Traffic, Volume 27, Issue 3, September 2026.
Human EHD protein subcellular localization. ABSTRACT Eps15 homology domain‐containing proteins comprise a conserved family of membrane‐remodeling ATPases that regulate endocytic trafficking, membrane fission, receptor recycling, primary ciliogenesis and membrane dynamics across eukaryotes. Since the initial identification of EHD1 and its Caenorhabditis
Devin Frisby, Gunjan Misri, Naava Naslavsky, Steve Caplan   +3 more
wiley   +1 more source

The response of rare CFTR mutations to specific modulator combinations

ERJ Open Research
Background The combination of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor (VX-445)–tezacaftor (VX-661)–ivacaftor (VX-770) (ETI) enables the effective rescue of CFTR function in people with the F508del mutation ...
Noemie Stanleigh, Michal Gur, Michal Shteinberg, Aryeh Weiss, Naama Sebbag-Sznajder, Deborah Duran, Myriam Grunewald, Liron Birimberg-Schwartz, Lea Pevzner, Ronen Bar-Yoseph, Jeffrey M. Beekman, Eitan Kerem, Michael Wilschanski, Batsheva Kerem   +13 more
doaj   +1 more source

Molecular Mechanisms of Electric Signals Generated at Corneal Wounds

The FASEB Journal, Volume 40, Issue 16, 31 August 2026.
Corneal injury triggers cell signaling (including calcium waves) leading to ion channel up‐regulation, increased ion pumping, and enhanced wound electric signals which stimulate wound healing. ABSTRACT Over 150 years ago, it was demonstrated that wounds produce electric fields and currents.
Brian Reid, Guillaume Luxardi, Min Zhao
wiley   +1 more source

Response to Elexacaftor/Tezacaftor/Ivacaftor in people with cystic fibrosis with the N1303K mutation: Case report and review of the literature

Heliyon
Cystic fibrosis (CF) is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Thousands of CFTR mutations have been identified, but only a fraction are known to cause CF, with the most common being the ...
Maria G. Tupayachi Ortiz, Nathalie Baumlin, Makoto Yoshida, Matthias Salathe   +3 more
doaj   +1 more source

Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators

Pediatric Pulmonology
AbstractIntroductionCystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non‐eligible patients for
Halime Nayır Büyükşahin, Nagehan Emiralioğlu, Ebru Yalçın, Velat Şen, Hadice Selimoğlu Şen, Hüseyin Arslan, Azer Kılıç Başkan, Fatma Betül Çakır, Cem Fırat Koray, Aslı İmran Yılmaz, Fatih Ercan, Derya Ufuk Altıntaş, Mahir Serbes, Özlem Keskin, Elif Arık, Figen Gülen, Meral Barlık, Oğuz Karcıoğlu, Ebru Damadoğlu, Mehmet Köse, Ali Ersoy, Ayşen Bingöl, Erdem Başaran, Eylül Pınar Çakır, Ayşe Tana Aslan, Yakup Canıtez, Merve Korkmaz, Ali Özdemir, Koray Harmancı, Şule Selin Soydaş, Melih Hangül, Hasan Yüksel, Gizem Özcan, Pervin Korkmaz, Mehmet Kılıç, Zeynep Gökçe Gayretli Aydın, Gönül Çaltepe, Demet Can, Sibel Doğru, Gökçen Kartal Öztürk, Ayşe Süleyman, Erdem Topal, Beste Özsezen, Mina Hızal, Ezgi Demirdöğen, Hamza Ogun, Şermin Börekçi, Hakan Yazan, Erkan Çakır, Tuğba Şişmanlar Eyüboğlu, Nazan Çobanoğlu, Güzin Cinel, Sevgi Pekcan, Uğur Özçelik, Deniz Doğru   +54 more
openaire   +5 more sources

Hospitalization Through Families’ Eyes: Comparing Inpatient Care Quality for Children With Sickle Cell Disease and Cystic Fibrosis in Canada

Pediatric Blood &Cancer, Volume 73, Issue 8, August 2026.
ABSTRACT Background Sickle cell disease (SCD) is a chronic, inherited hemoglobinopathy that requires frequent hospitalization for disease‐related complications. Canadian data on inpatient care is limited. This study compared caregiver‐reported hospital experiences of children with SCD to those with cystic fibrosis (CF), a chronic, autosomal recessive ...
Hailey M. Zwicker, Kyle Kemp, Perri Tutelman, Sharon H. J. Hou, Paul Fairie, Maria Santana, Gregory M. T. Guilcher, Taryn Fay‐McClymont, Glenda Bendiak, Wendy Pelletier, Nicola Wright, Fiona Schulte   +11 more
wiley   +1 more source

Pathogenetic treatment of cystic fibrosis: expanding access to drugs

Лечащий Врач
Background. Cystic fibrosis is a hereditary monogenic disease inherited in an autosomal recessive pattern, caused by a mutation in the cystic fibrosis transmembrane regulator (CFTR) gene and characterized by multisystem involvement of exocrine glands ...
S. N. Avdeev, Z. M. Merzhoeva, V. V. Gaynitdinova, E. L. Amelina   +3 more
doaj   +1 more source

Uncovering Cystic Fibrosis Carrier: Insights From a Heterozygous CFTR‐F508del Rabbit Model

International Forum of Allergy &Rhinology, Volume 16, Issue 8, Page 788-796, August 2026.
ABSTRACT Background Chronic rhinosinusitis (CRS) is a heterogeneous inflammatory disorder frequently associated with impaired mucociliary clearance and bacterial infection. Individuals carrying a single cystic fibrosis transmembrane conductance regulator (CFTR) mutation exhibit partial CFTR dysfunction and are increasingly recognized as being at risk ...
Do‐Yeon Cho, Alexis E. McFeely, Daniel Skinner, Shaoyan Zhang, Justin Hedvat, Weslee Ping‐ay, Daniel M. Beswick, Justin H. Turner, Bradford A. Woodworth, Jie Xu   +9 more
wiley   +1 more source

Successful Rapid Desensitization to Ceftazidime/Avibactam in a Patient With Cystic Fibrosis and Multidrug‐Resistant Pseudomonas aeruginosa Pneumonia: A Case Report

Clinical Case Reports, Volume 14, Issue 8, August 2026.
ABSTRACT Ceftazidime/avibactam (CAZ‐AVI) is an important therapeutic option for multidrug‐resistant (MDR) Pseudomonas aeruginosa infections; however, hypersensitivity reactions may preclude its use and create significant therapeutic challenges. We report a 21‐year‐old woman with cystic fibrosis and recurrent MDR P.
Zuoren Zhou, Jinqi Zhu, Ge Wu, Xi Kang, Qing Wang, Jie Liu, Yan Jiang   +6 more
wiley   +1 more source