Results 141 to 150 of about 1,459,532 (261)

A Non‐Channel Function of CFTR: Attenuating Mitochondrial Oxidative Stress and Cardiomyocyte Senescence via Stabilization by USP45

Aging Cell, Volume 25, Issue 7, July 2026.
USP45 stabilizes CFTR through deubiquitination, thereby activating PMCA, which inhibits calcium/chloride overload and mitochondrial oxidative stress in senescent cardiomyocytes, ultimately alleviating cardiomyocyte senescence. ABSTRACT Cardiomyocyte senescence drives cardiovascular disease, underscoring the need to define its molecular mechanisms.
Chun Chen, Longtan Jiang, Yuewen Qiu, Chong Liu, Xiao Long, Pengcui Wu, Liang Li, Haixia Xu, Ping Deng, Li Yang, Qiao Jin   +10 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

Microbial Metabolite‐Stimulated Bitter Taste Receptor T2R14 Signaling Is Modulated by CFTR Interactions

The FASEB Journal, Volume 40, Issue 11, 15 June 2026.
Bitter taste receptors (T2Rs) play an important role in physiological and cellular responses. Bitter tasting chemicals and microbial metabolites can bind to T2R14, which enables interactions between T2R14 and specific intracellular domains of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel.
Tejas Gupte, Nisha Singh, Vikram Bhatia, Kavisha Arora, Shayan Amiri, Paul Fernyhough, Anjaparavanda P. Naren, Shyamala Dakshinamurti, Prashen Chelikani   +8 more
wiley   +1 more source

Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey.

, 2020
BACKGROUND As their long-term prognosis improves, women with CF are increasingly choosing to have children, but the safety of CFTR modulators in pregnancy and breastfeeding is currently unknown.
Taylor-Cousar, Jennifer L, Nash, Edward F, Middleton, Peter G   +2 more
core   +1 more source

Clinical pharmacology of CFTR modulators

With the development of cystic fibrosis transmembrane receptor (CFTR) modulating drugs, the landscape in cystic fibrosis (CF) care has changed dramatically. These drugs enable the treatment of the underlying cause of the disease. Although CFTR modulators show an impressive clincal effect at group level in people with CF (pwCF) with specific mutations ...
Meer - van de Grint, Renske van der, Heijerman, H.G.M., Wilms, E.B.   +2 more
openaire   +3 more sources

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

Respiratory Organ‐on‐a‐Chip for Disease Modeling: From Architecture to Functional Integration

Advanced Healthcare Materials, Volume 15, Issue 23, 19 June 2026.
Respiratory organ‐on‐a‐chip (ROC) models capture key mechanical and cellular cues of the human respiratory system, enabling quantitative dissection of disease mechanisms. This review links ROC architectures to disease modeling, functional integration, and commercialization, and proposes a decision framework that aligns model complexity with mechanistic
Jinzhuo Hu, Yongjie Tang, Sidi Liu, Tao Xu, Qian Liu   +4 more
wiley   +1 more source

Clinical Outcomes in Patients with Cystic Fibrosis Receiving CFTR Modulators: A Comparison of Childhood Versus Adolescent Initiation

Children
Background/objectives: Cystic fibrosis (CF) is a life-limiting genetic disorder affecting multiple organ systems. This study compared clinical outcomes, hospitalization rates, and survival between children and adolescents with CF who received CFTR ...
Eman A. Toraih, Hassan A. Malik, Rahib K. Islam, Humza A. Pirzadah, Ahmed Abdelmaksoud, Rami M. Elshazli, Paul Antwi Boasiako, Shehab Ahmed Alenazi, Angelique Dabel, Jessan A. Jishu, Bandar T. Alenezi, Hani Aiash, Manal S. Fawzy   +12 more
doaj   +1 more source

Long‐term impact of Elexacaftor/Tezacaftor/ivacaftor on pulmonary, nutritional and metabolic outcomes in homozygous F508del cystic fibrosis patients: A real‐world cohort study

British Journal of Clinical Pharmacology, Volume 92, Issue 6, Page 1822-1832, June 2026.
Aim Elexacaftor/tezacaftor/ivacaftor (ETI) has markedly improved cystic fibrosis (CF) outcomes. However, its long‐term impact on nutrition, metabolism and liver health remains underexplored. We assessed 30‐month changes in pulmonary, nutritional, metabolic and inflammatory markers in people with CF (PwCF) homozygous for F508del.
Nicola Perrotta, Luigi Angelo Fiorito, Roberta Vescovo, Anna Virgilio, Giulia Amato, Giuseppe Cimino   +5 more
wiley   +1 more source

Personalizing CFTR modulator therapies

, 2020
over 2000 genetic mutations that can cause CF, resulting in many phenotypes. Recently new drugs were developed that treat the disease at the origin of the problem; they enhance the function of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein which is not functioning properly in CF. These potentially life saving drugs are currently
Berkers, Gitte, Ent, C.K. van der, Beekman, J.M.   +2 more
openaire   +2 more sources