Disruption of Interleukin-1β Autocrine Signaling Rescues Complex I Activity and Improves ROS Levels in Immortalized Epithelial Cells with Impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function [PDF]
Patients with cystic fibrosis (CF) have elevated concentration of cytokines in sputum and a general inflammatory condition. In addition, CF cells in culture produce diverse cytokines in excess, including IL-1B. We have previously shown that IL-1B, at low
Clauzure, Mariangeles +5 more
core +1 more source
Temperature-dependent release of ATP from human erythrocytes: Mechanism for the control of local tissue perfusion [PDF]
Copyright @ 2012 The AuthorsThis article has been made available through the Brunel Open Access Publishing Fund.Human limb muscle and skin blood flow increases significantly with elevations in temperature, possibly through physiological processes that ...
Abraham +58 more
core +2 more sources
Cystic Fibrosis Polymorphic Variants in a Russian Population
Anna Kiseleva,1,* Marina Klimushina,1,* Evgeniia Sotnikova,1,* Olga Skirko,1 Mikhail Divashuk,1,2 Olga Kurilova,1 Alexandra Ershova,1 Eleonora Khlebus,1 Anastasia Zharikova,1,3,4 Irina Efimova,1 Maria Pokrovskaya,1 Petr A Slominsky,5 Svetlana Shalnova,1 ...
Kiseleva A +14 more
doaj
Chronic infection and inflammation are the primary causes of declining lung function in Cystic Fibrosis (CF) patients. ORKAMBI® (Lumacaftor-Ivacaftor) is an approved combination therapy for Cystic Fibrosis (CF) patients bearing the most common ...
Onofrio Laselva +3 more
doaj +1 more source
CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells
People with cystic fibrosis should be considered at increased risk of developing severe symptoms of COVID-19. Strikingly, a broad array of evidence shows reduced spread of SARS-CoV-2 in these subjects, suggesting a potential role for CFTR in the ...
Virginia Lotti +12 more
doaj +1 more source
Bioengineered 3D hPSC‐Cholangiocyte Ducts With Physiological Signals for Biliary Disease Modeling
Tian and colleagues generated a bioengineered bile duct from human pluripotent stem cell (hPSC)‐derived intrahepatic cholangiocytes within a high‐throughput, 384‐well platform to systematically examine the influence of biliary physiological signals including fluid flow, stromal cells and bile acids, and models intrahepatic biliary disease progression ...
Britney Tian +10 more
wiley +1 more source
Objective: Cystic fibrosis (CF) is a congenital condition caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. CF-related diabetes (CFRD) is a common comorbidity among people with CF (pwCF) and is associated with increased ...
Anna Edlund +3 more
doaj +1 more source
Cystic fibrosis, molecular genetics for all life
Cystic fibrosis (CF) is the most frequent lethal autosomal recessive disorder among Caucasians (incidence: 1:2,500 newborn). In the last two decades CF prognosis considerably improved and many patients well survive into their adulthood.
Ausilia Elce +5 more
doaj +1 more source
Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused by a defined subset of minimal function CFTR mutants. [PDF]
Available CFTR modulators provide no therapeutic benefit for cystic fibrosis (CF) caused by many loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel, including N1303K. We previously introduced the
Finkbeiner, Walter E +7 more
core
Respiratory Organ‐on‐a‐Chip for Disease Modeling: From Architecture to Functional Integration
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 +4 more
wiley +1 more source

