Results 91 to 100 of about 1,105,613 (399)

VX‐659–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

New England Journal of Medicine, 2018
BACKGROUND The next‐generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector VX‐659, in triple combination with tezacaftor and ivacaftor (VX‐659–tezacaftor–ivacaftor), was developed to restore the function of Phe508del CFTR protein
J. Davies, S. Moskowitz, C. Brown, A. Horsley, M. Mall, E. McKone, B. Plant, D. Prais, B. Ramsey, J. Taylor-Cousar, E. Tullis, A. Uluer, C. Mckee, S. Robertson, R. Shilling, C. Simard, F. van Goor, D. Waltz, F. Xuan, T. Young, S. Rowe   +20 more
semanticscholar   +1 more source

Specific Antibodies to Recombinant Allergens of \u3cem\u3eAspergillus fumigatus\u3c/em\u3e in Cystic Fibrosis Patients with ABPA [PDF]

, 2006
Background Aspergillus fumigatus, a widely distributed fungus, has been implicated in causing life threatening infections as well as severe asthma and allergic diseases in man.
Bansal, Naveen K., Knutsen, Alan P., Kurup, Viswanath P., Moss, Richard B.   +3 more
core   +1 more source

A new targeted CFTR mutation panel based on next-generation sequencing technology [PDF]

, 2017
Searching for mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) is a key step in the diagnosis of and neonatal and carrier screening for cystic fibrosis (CF), and it has implications for prognosis and personalized therapy ...
Alberti, Luisella, Biffignandi, Alice, Bruno, Sabina Maria, Colombo, Carla, Corbetta, Carlo, Costantino, Lucy, Giannone, Valentina, Lucarelli, Marco, Porcaro, Luigi, Seia, Manuela, Torresani, Erminio   +10 more
core   +2 more sources

Developmental Hypoxia Enhances Kidney Organoid Complexity and Maturity

Advanced Science, EarlyView.
The current bottleneck in metanephric mesenchyme (MM)‐derived nephron organoid development is the absence of a connected network of collecting ducts (CD) derived from the ureteric bud (UB). To address this limitation, this study proposes a hypoxia‐based differentiation method that mimics the natural renal developmental environment, enabling the ...
Hyeonji Lim, Dohui Kim, Haejin Yoon, Joo H. Kang, Yong Jun Kim, Dong Sung Kim, Tae‐Eun Park   +6 more
wiley   +1 more source

A medium composition containing normal resting glucose that supports differentiation of primary human airway cells

Scientific Reports, 2022
Primary cells isolated from the human respiratory tract are the state-of-the-art for in vitro airway epithelial cell research. Airway cell isolates require media that support expansion of cells in a basal state to maintain the capacity for ...
Rachel Morgan, Candela Manfredi, Kristen F. Easley, Lionel D. Watkins, William R. Hunt, Steven L. Goudy, Eric J. Sorscher, Michael Koval, Samuel A. Molina   +8 more
doaj   +1 more source

Systematic genetic analysis of the MHC region reveals mechanistic underpinnings of HLA type associations with disease. [PDF]

, 2019
The MHC region is highly associated with autoimmune and infectious diseases. Here we conduct an in-depth interrogation of associations between genetic variation, gene expression and disease.
Aguiar, Assis, Auton, Bijvelds, Blackwell, Bonder, D'Antonio, D'Antonio-Chronowska, DeBoever, Dechecchi, DeGiorgio, Dendrou, Diwakar, Dobin, Eguchi, Ernst, Fehrmann, Freudenberg, Gambino, Gensterblum-Miller, Giambartolomei, González-Galarza, Gough, Graffelman, Guo, Hardy, Harrow, Herrmann, Holoshitz, Huang, Jakubosky, Jakubosky, Jensen, Jia, Kilpinen, Kilpinen, Klein, Kontakioti, Kundaje, Laki, Lam, Lee, Leung, Li, Li, Li, Li, Li, Lyczak, Mahdi, Mall, Matzaraki, Mayba, McNicholas, Miretti, Morison, Munder, Nariai, Norman, Oldstone, Panopoulos, Panousis, Pier, Robinson, Sondo, Stegle, Stoltz, Streeter, Tan, Tomati, Trowsdale, Van der Auwera, Vicente, Wilke, Yin, Zhang, Zheng   +76 more
core   +2 more sources

Macrophage‐Mediated Transport of Insoluble Indirubin Induces Hepatic Injury During Intestinal Inflammation

Advanced Science, EarlyView.
Plant‐derived bioactive molecules with low solubility and permeability induce hepatocyte injury, though the mechanisms driving their hepatic effects remain poorly understood. This study identifies a novel transport pathway in which poorly soluble indirubin accumulates in the liver via macrophage‐mediated uptake in Peyer's patches, exacerbating hepatic ...
Yiqi Xu, Jingchun Shi, Heung‐Lam Mok, Cheng Lyu, Junbang Chen, Chunhua Huang, Hongyan Qin, Chengyuan Lin, Hor‐Yue Tan, Zhaoxiang Bian   +9 more
wiley   +1 more source

The NSAID glafenine rescues class 2 CFTR mutants via cyclooxygenase 2 inhibition of the arachidonic acid pathway

Scientific Reports, 2022
Most cases of cystic fibrosis (CF) are caused by class 2 mutations in the cystic fibrosis transmembrane regulator (CFTR). These proteins preserve some channel function but are retained in the endoplasmic reticulum (ER).
Graeme W. Carlile, Qi Yang, Elizabeth Matthes, Jie Liao, Véronique Birault, Helen F. Sneddon, Darren L. Poole, Callum J. Hall, John W. Hanrahan, David Y. Thomas   +9 more
doaj   +1 more source

Inflammation in cystic fibrosis: An update

Pediatric Pulmonology, 2018
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease progression making it an active area of research and important therapeutic target.
E. Roesch, D. Nichols, J. Chmiel
semanticscholar   +1 more source

Anticodon Engineered Transfer RNA (tRNASUAG) Inhibits Hepatitis B Virus Replication by Promoting the Degradation of Core Protein

Advanced Science, EarlyView.
This study employed tRNASUAG, an anticodon‐engineered tRNA (ACE‐tRNA), to introduce a phosphorylatable serine into the C‐terminal domain of HBc by reading through its stop codon, thereby potentially promoting the ubiquitin‐proteasome degradation of HBc, and subsequently inhibiting HBV replication. Through a tRNASUAG‐gHBV1‐tRNASUAG‐gHBV2‐tRNASUAG tandem
Xingwen Yang, Huiying Sun, Ziheng Luo, Qinxin Zhang, Yarong Song, Jie Li, Xiaoyun Liu, Jie Wang   +7 more
wiley   +1 more source