Results 61 to 70 of about 11,543 (240)

CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011–2018

ERJ Open Research, 2022
Background People with cystic fibrosis are at increased risk of pulmonary nontuberculous mycobacteria (NTM) disease. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are associated with reduced lung infection with pathogens like ...
Emily E. Ricotta, D. Rebecca Prevots, Kenneth N. Olivier   +2 more
doaj   +1 more source

Identifying transcription factors controlling the basal expression of human MRP4 highlights a substantial role for Sp1

FEBS Open Bio, EarlyView.
The MRP4 transporter exports several drugs and signaling molecules. Here, we identified key promoter elements regulating basal MRP4 expression. Using reporter assays, we defined a conserved region with essential Sp1 and contributory Ets sites, which controlled basal MRP4 expression.
Debora Singer, Angelique Kragl, Katrin Ziems, Juliane Glaubitz, Sophie Grammbauer, Susanne Hildebrandt, Mladen V. Tzvetkov, Gabriele Jedlitschky   +7 more
wiley   +1 more source

The Ubiquitin Ligase RNF34 Participates in the Peripheral Quality Control of CFTR (RNF34 Role in CFTR PeriQC)

Frontiers in Molecular Biosciences, 2022
The peripheral protein quality control (periQC) system eliminates the conformationally defective cystic fibrosis transmembrane conductance regulator (CFTR), including ∆F508-CFTR, from the plasma membrane (PM) and limits the efficacy of pharmacological ...
Shogo Taniguchi, Yukiko Ito, Hibiki Kiritani, Asuka Maruo, Ryohei Sakai, Yuji Ono, Ryosuke Fukuda, Tsukasa Okiyoneda   +7 more
doaj   +1 more source

Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused by a defined subset of minimal function CFTR mutants. [PDF]

, 2019
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, Haggie, Peter M, Nielson, Dennis W, Phuan, Puay-Wah, Rivera, Amber A, Tan, Joseph-Anthony, Verkman, Alan S, Zlock, Lorna   +7 more
core  

Interplay between CFTR Phosphorylation, CFTR-ATPase Activity, and Anion Flux [PDF]

, 2016
The cystic fibrosis (CF) transmembrane conductance regulator (CFTR, ABCC7), mutations of which cause CF, belongs to the ATP binding cassette (ABC) transporter family and works as a channel for small anions, such as chloride and bicarbonate. Anion channel
Esposito, Cinzia, Hellstern, Manuel, Seelig, Anna, Zwick, Matthias   +3 more
core   +1 more source

Bioengineered 3D hPSC‐Cholangiocyte Ducts With Physiological Signals for Biliary Disease Modeling

Advanced Healthcare Materials, EarlyView.
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, Mina Ogawa, Manami Kondo, Gabrielle Langeveld, Ling Jun Huan, Feng Zhang, Andrew Hollinger, Sara Deir, Christine Bear, Boyang Zhang, Shinichiro Ogawa   +10 more
wiley   +1 more source

CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages

Frontiers in Cellular and Infection Microbiology, 2022
Cystic fibrosis (CF) human and mouse macrophages are defective in their ability to clear bacteria such as Burkholderia cenocepacia. The autophagy process in CF (F508del) macrophages is halted, and the underlying mechanism remains unclear.
Asmaa Badr, Asmaa Badr, Mostafa Eltobgy, Kathrin Krause, Kathrin Krause, Kaitlin Hamilton, Shady Estfanous, Shady Estfanous, Kylene P. Daily, Arwa Abu Khweek, Arwa Abu Khweek, Ahmad Hegazi, Midhun N. K. Anne, Cierra Carafice, Frank Robledo-Avila, Youssra Saqr, Xiaoli Zhang, Tracey L. Bonfield, Mikhail A. Gavrilin, Santiago Partida-Sanchez, Stephanie Seveau, Estelle Cormet-Boyaka, Amal O. Amer   +22 more
doaj   +1 more source

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity. [PDF]

, 2019
Deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is the most frequent mutation causing cystic fibrosis (CF). F508del-CFTR is misfolded and prematurely degraded. Recently thymosin a-1 (
Armirotti, Andrea, Braccia, Clarissa, Cholon, Deborah M, Galietta, Luis JV, Gentzsch, Martina, Guidone, Daniela, Hanrahan, John W, Lukacs, Gergely L, Matthes, Elizabeth, Pedemonte, Nicoletta, Phuan, Puay-Wah, Tomati, Valeria, Veit, Guido, Verkman, Alan S   +13 more
core  

Biology of Cholangiocytes: From Bench to Bedside [PDF]

, 2016
Cholangiocytes, the lining epithelial cells in bile ducts, are an important subset of liver cells. They are activated by endogenous and exogenous stimuli and are involved in the modification of bile volume and composition.
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core   +1 more source

Strategies to investigate the mechanism of action of CFTR modulators [PDF]

Journal of Cystic Fibrosis, 2004
The malfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel is associated with a wide spectrum of disease. In the search for modulators of CFTR, pharmaceutical agents have been identified that (i) act indirectly by regulating the protein kinases and phosphatases, which control CFTR, and (ii) interact directly with CFTR.
Cai, Zhiwei, Scott-Ward, Toby S., Li, Hongyu, Schmidt, André, Sheppard, David N.   +4 more
openaire   +2 more sources