Results 91 to 100 of about 6,532 (197)

Activity of innate antimicrobial peptides and ivacaftor against clinical cystic fibrosis respiratory pathogens [PDF]

, 2017
There is a clear need for new antimicrobials to improve current treatment of chronic lung infection in people with cystic fibrosis (CF). This study determined the activities of antimicrobial peptides (AMPs) and ivacaftor, a novel CF transmembrane ...
Dubois, Alice V, Taggart, Clifford C, Michael M. Tunney, Elborn, J Stuart, Payne, Joanna E, Ingram, Rebecca J, Sinead Weldon, J. Stuart Elborn, Clifford C. Taggart, Tunney, Michael M, Alice V. Dubois, Weldon, Sinead, Joanna E. Payne, Rebecca J. Ingram   +13 more
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Ivacaftor attenuates gentamicin-induced ototoxicity through the CFTR-Nrf2-HO1/NQO1 pathway

Redox Report
Objectives Gentamicin is one of the most common ototoxic drugs that can lower patients' quality of life. Oxidative stress is a key factors inducing sensory hair cell death during gentamicin administration.
Rui Hu, Fan Wu, Yi-Qing Zheng
doaj   +1 more source

Images of C-sweat responses for CF subject 8: male, I507del/R117H-5T (±) ivacaftor (N of 1 trial).

, 2014
S8 was tested 3 times (−) ivacaftor and 5 times (+) ivacaftor. Only the first 3 (+) ivacaftor tests are shown. (A-C) (−) ivacaftor; (D-F) (+) ivacaftor. Each image shows the field 30 min after the cocktail injection.
Jessica E. Char (476386), Hyung-ju Cho (5656717), Ewart A. C. Thomas (476393), Colleen Dunn (476388), Il-Ho Park (476392), Richard B. Moss (521193), Jeffrey J. Wine (207605), Carlos Milla (476390), Zoe Davies (521192), Eric Frisbee (476389), Marlene H. Wolfe (476396), Jin Hyeok Jeong (521191)   +11 more
core   +1 more source

Ivacaftor pharmacokinetics and lymphatic transport after enteral administration in rats

Frontiers in Pharmacology
Background: Ivacaftor is a modern drug used in the treatment of cystic fibrosis. It is highly lipophilic and exhibits a strong positive food effect.
Jiří Pozniak, Pavel Ryšánek, David Smrčka, Petr Kozlík, Tomáš Křížek, Jaroslava Šmardová, Anežka Nováková, Debanjan Das, Daniel Bobek, Mahak Arora, Jiří Hofmann, Tereza Doušová, Martin Šíma, Ondřej Slanař   +13 more
doaj   +1 more source

Data from: The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: human in vivo measurements

, 2017
We optically measured effects of orally available ivacaftor (Kalydeco®) on sweat rates of identified glands in 3 R117H subjects, each having a unique set of additional mutations, and compared them with 5 healthy control subjects tested contemporaneously.
Milla, Carlos, Davies, Zoe, Moss, Richard B., Dunn, Colleen, Char, Jessica E., Wine, Jeffrey J.   +5 more
core   +1 more source

VX-445-tezacaftor-ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles

, 2018
BACKGROUND : VX-445 is a next-generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore Phe508del CFTR protein function in patients with cystic fibrosis when administered with tezacaftor and ivacaftor (VX-445 ...
Moskowitz, Samuel M, Ramsey, Bonnie W, Tullis, Elizabeth, Van Goor, Fredrick, Young, Tim, VX16-445-001 study group, for the, Marigowda, Gautham, Robertson, Sarah, Van Braeckel, Eva, McKee, Charlotte M, Mall, Marcus A, Taylor-Cousar, Jennifer L, Rowe, Steven M, Simard, Christopher, Waltz, David, Xuan, Fengjuan, Keating, Dominic, Burr, Lucy, Savage, Jessica, McKone, Edward F, Daines, Cori, Sass, Laura A   +21 more
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Effectivenesss of ivacaftor in severe cystic fibrosis patients and non-G551D gating mutations

, 2019
Background: Ivacaftor is a significant innovation in the treatment of cystic fibrosis (CF) with gating mutations. A substantial percentage of patients with CF have severe lung involvement, but these patients are usually excluded from phase III clinical ...
Francalanci M., Lucidi V., Vitullo P., Iacotucci P., Leonetti G., Colangelo C., Carnovale V., Ferrara N., Castellani C., Salvatore D., Braggion C., Cimino G., Manca A.   +12 more
core   +1 more source

The potentiator ivacaftor is essential for pharmacological restoration of F508del-CFTR function and mucociliary clearance in cystic fibrosis

JCI Insight
Pharmacological rescue of F508del-CFTR by the triple combination CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) leads to unprecedented clinical benefits in patients with cystic fibrosis (CF).
Anita Balázs, Tihomir Rubil, Christine K. Wong, Jasmin Berger, Marika Drescher, Kathrin Seidel, Mirjam Stahl, Simon Y. Graeber, Marcus A. Mall   +8 more
doaj   +1 more source

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

M- and C-sweat secretion (±) ivacaftor for CF subject 2: female, F508del/G551D.

, 2014
(A) M-sweat responses were unchanged by ivacaftor in this subject. Each point is log transformed values for a single pre-test and mean of 2 post-test measures for each of 87 glands. Mean volumes were 28.2±12.5 vs. 31.9±14.6 nl−gland, pre and post, n = 87
Jessica E. Char (476386), Hyung-ju Cho (5656717), Ewart A. C. Thomas (476393), Colleen Dunn (476388), Il-Ho Park (476392), Richard B. Moss (521193), Jeffrey J. Wine (207605), Carlos Milla (476390), Zoe Davies (521192), Eric Frisbee (476389), Marlene H. Wolfe (476396), Jin Hyeok Jeong (521191)   +11 more
core   +1 more source