Results 101 to 110 of about 9,731 (210)

Molecular basis of cystic fibrosis and modern therapeutic approaches [PDF]

, 2019
Identifikacija molekularne povezanosti između genske nefunkcionalnosti CFTR gena te patofiziološke i kliničke pozadine cistične fibroze omogućila je razvoj suvremenih terapijskih modela koji su primarno utemeljeni na individualiziranom pristupu ...
Dragičević, Dorian
core   +2 more sources

Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR [PDF]

, 2018
Atkinson, Jeffrey, Barto, Tara Lynn, Haddad, Tarik, Jain, Manu, Marigowda, Gautham, Pilewski, Joseph, Tang, Rui, Taylor-Cousar, Jennifer L, Tian, Simon, VH14-809-106 Investigator Group,, Waltz, David   +10 more
core   +2 more sources

Impact of lumacaftor/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden

Therapeutic Advances in Respiratory Disease
Background: A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden.
Mahasin Al Shakirchi, Kimmo Sorjonen, Lena Hjelte, Lena Klingspor, Peter Bergman, Petrea Ericson, Marcus Svedberg, Ulrika Lindberg, Christine Hansen, Isabelle de Monestrol   +9 more
doaj   +1 more source

In Vitro Restoration of Colistin Susceptibility by Ivacaftor Synergy with Limited Reproducibility in a Murine Pneumonia Model

Antibiotics
Background: We aimed to investigate the potential synergistic effect of ivacaftor combined with colistin against Pseudomonas aeruginosa and Klebsiella pneumoniae, and to elucidate the underlying molecular mechanisms through metabolomic analysis and its ...
Ana Verónica Halperin, Franziska Schwartz, Lars Christophersen, José Pérez-del Palacio, Manuel Ponce-Alonso, José Avendaño-Ortiz, Juan de Dios Caballero, Rafael Cantón, Claus Moser, Rosa del Campo   +9 more
doaj   +1 more source

CFTR and ENaC functions in cystic fibrosis [PDF]

, 2014
La fibrosis quística se debe a la ausencia o defecto del canal transmembrana regulador de la fibrosis quística (CFTR), un canal de cloruro codificado en el gen cftr que juega un papel clave en la homeostasis del agua e iones.
Kotsias, Basilio Aristides, Marino, Gabriela Inés, Palma, Alejandra Graciela   +2 more
core  

Lumacaftor-ivacaftor in the treatment of cystic fibrosis: design, development and place in therapy

Drug Design, Development and Therapy, 2019
GJ ConnettNational Institute for Health Research, Southampton Respiratory Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust, Southampton, SO16 6YD, UKAbstract: Lumacaftor-ivacaftor is a combination of two small molecule ...
Connett GJ
doaj  

Altered functional interactions between CFTR disease mutants ΔF508 and G551D and the protein kinase A catalytic subunit. [PDF]

J Physiol
Závoti O, Simon MA, Csanády L.
europepmc   +1 more source

Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup [PDF]

, 2016

core   +1 more source

Cystic Fibrosis: Breakthrough Drugs at Break-the-Bank Prices. [PDF]

, 2015
O'Sullivan, Brian P, Orenstein, David M, Quinton, Paul M   +2 more
core   +1 more source

Systematic Review of Nasal Endoscopy Scores in Cystic Fibrosis Patients Treated With Cystic Fibrosis Transmembrane Conductance Regulator Modulators. [PDF]

Pediatr Pulmonol
Gallardo R, Silva BS, Salgado LS, Ribeiro JD, Sakano E.   +4 more
europepmc   +1 more source