Results 111 to 120 of about 5,816 (202)

Human Nasal and Bronchial Epithelium in Cystic Fibrosis: Differences in Expression of Airway pH Regulatory Proteins

Pediatric Pulmonology, Volume 61, Issue 8, August 2026.
ABSTRACT Background Decreased apical bicarbonate transport into the airway surface liquid (ASL) has been associated with decreased ASL pH, which can have adverse respiratory effects. However, the human CF epithelium can normalize ASL pH. Thus, we hypothesized that pH regulatory proteins other than the CFTR could be altered in the CF epithelium ...
Michael D. Davis, Laura Smith, Yi Zhao, Rania Abdul Rahman, Faiza Khalid, James Chmiel, Katie Boyne, Ivana A. Daniels, Benjamin Gaston, Nadzeya Marozkina   +9 more
wiley   +1 more source

In utero and postnatal ivacaftor/lumacaftor therapy rescues multiorgan disease in CFTR-F508del ferrets

JCI Insight
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, with F508del being the most prevalent mutation.
Idil Apak Evans, Xingshen Sun, Bo Liang, Amber R. Vegter, Lydia Guo, Thomas J. Lynch, Yan Zhang, Yulong Zhang, Yaling Yi, Yu Yang, Zehua Feng, Soo Yeun Park, Amanita Shonka, Hannah McCumber, Lisi Qi, Peipei Wu, Guangming Liu, Allison Lacina, Kai Wang, Katherine N. Gibson-Corley, David K. Meyerholz, Dominique H. Limoli, Bradley H. Rosen, Ziying Yan, Douglas J. Bartels, John F. Engelhardt   +25 more
doaj   +1 more source

Development and analytical validation of a targeted short‐read next generation sequencing‐based pharmacogenetic panel for comprehensive variant detection

British Journal of Pharmacology, Volume 183, Issue 16, Page 4735-4760, August 2026.
Abstract Background and Purpose Genomic profiling of patients for genetic variants that modify the effect of specific medications has many benefits, including the possibility of avoiding toxicities and ensuring an adequate effect of the medication. Our intention was to develop a comprehensive, high‐quality pharmacogenetic test panel for clinical use ...
Anna Gréen, Nils Johan Fredriksson, Michael Andresen, Jon Jonasson, Malin Lindqvist Appell, Henrik Gréen   +5 more
wiley   +1 more source

Identification of drug repurposing candidates for the treatment of polycystic kidney disease

British Journal of Pharmacology, Volume 183, Issue 16, Page 5082-5101, August 2026.
Background and Purpose Autosomal dominant polycystic kidney disease (ADPKD) is a leading cause of end‐stage renal disease with limited treatment options. Drug repurposing offers a promising strategy to find effective treatments. Experimental Approach We identified birinapant, bardoxolone methyl and salicylic acid as repurposing candidates for ADPKD and
Alina Meyer, Paraskevi Sotiropoulou, Koussai Bouker, Dennis Svedberg, Rebekkah Hammar, Hester Bange, Per Larsson, André Mateus, Daniel R. Dietrich, Per Artursson   +9 more
wiley   +1 more source

Profile of tezacaftor/ivacaftor combination and its potential in the treatment of cystic fibrosis

Therapeutics and Clinical Risk Management, 2019
Dejene Shiferaw,* Shoaib Faruqi*Department of Respiratory Medicine, Hull University Teaching Hospitals NHS Trust, Cottingham HU16 5JQ, UK *These authors contributed equally to this workCorrespondence: Shoaib FaruqiDepartment of Respiratory Medicine ...
Shiferaw D, Faruqi S
doaj  

Triple combination cystic fibrosis transmembrane receptor modulator effects on glycaemia and insulin kinetics in cystic fibrosis with and without diabetes

Internal Medicine Journal, Volume 56, Issue 8, Page 1356-1360, August 2026.
Abstract Background Greater insight into the effects of cystic fibrosis (CF) transmembrane modulators such as elexacaftor‐tezacaftor‐ivacaftor (ETI) on glucose metabolism can support a more dynamic and individualised approach to CF‐related dysglycaemia.
Yi W. Chen, Rachel Prasad, Peter G. Middleton, D. Jane Holmes‐Walker   +3 more
wiley   +1 more source

Integrated histopathological, physiological, biochemical, and molecular analysis of gill responses to acute salinity stress in red tilapia (Oreochromis spp.)

Journal of the World Aquaculture Society, Volume 57, Issue 4, August 2026.
Abstract Growing freshwater scarcity in China is driving aquaculture interests toward saline‐tolerant fish species. Red tilapia (Oreochromis spp.) is a euryhaline species with strong osmoregulatory capacity, making it an ideal model for investigating stress‐induced physiological changes.
Moustafa Saleh, Rahma Aboueleila, Mohamed Fekri Badran, Jixiang Hua, Yalun Dong, Siqi Lu, Wen‐jing Tao, Yifan Tao, Jun Qiang   +8 more
wiley   +1 more source

Prevalence of Overweight and Obesity in the Era of CFTR Modulators in Patients with Cystic Fibrosis. [PDF]

Nutrients
Bashir A, Kastl MB, Wang X, Padula L, Reid E, Kofsky R, Pai N, Mascarenhas M.   +7 more
europepmc   +1 more source

Impact of COVID-19 and CFTR Modulators on Cystic Fibrosis: A Real-World Analysis of Care Patterns. [PDF]

Pediatr Pulmonol
Hinton AC, Sears EH, Lopez-Pintado S, Zuckerman JB.   +3 more
europepmc   +1 more source

Beyond BMI: Nutritional Recovery and Functional Implications of CFTR Modulators in Cystic Fibrosis. [PDF]

Biology (Basel)
Linguiti G, Granberg V, Leonetti G, Lassandro G, Lassandro M, Delvecchio M, Giordano P.   +6 more
europepmc   +1 more source