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https://www.readbyqxmd.com/read/28433526/orkambi-in-patients-with-severe-disease-bumps-in-the-road-to-cftr-modulation
#1
EDITORIAL
Alex Horsley, Peter Barry
No abstract text is available yet for this article.
April 19, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28431985/quercetin-interferes-with-the-fluid-volume-and-receptivity-development-of-the-uterus-in-rats-during-the-peri-implantation-period
#2
Huma Shahzad, Nelli Giribabu, Kamarulzaman Karim, Normadiah Kassim, Sekaran Muniandy, Kilari Eswar Kumar, Naguib Salleh
HYPOTHESIS: quercetin could induce changes to the fluid volume and receptivity development of the uterus during peri-implantation period. METHODS: Female rats were treated with quercetin (10, 25 and 50mg/kg/day) subcutaneously beginning from day-1 pregnancy. Uterus was harvested at day-4 (following three days quercetin treatment) for morphological, ultra-structural, protein and mRNA expressional changes and plasma sex-steroid levels analyses. In another cohort of rats, implantation rate was determined at day-6 (following five days quercetin treatment)...
April 18, 2017: Reproductive Toxicology
https://www.readbyqxmd.com/read/28428011/cftr-dependent-defect-in-alternatively-activated-macrophages-in-cystic-fibrosis
#3
Abdullah A Tarique, Peter D Sly, Patrick G Holt, Anthony Bosco, Robert S Ware, Jayden Logan, Scott C Bell, Claire E Wainwright, Emmanuelle Fantino
BACKGROUND: The role of the macrophages in cystic fibrosis (CF) lung disease has been poorly studied. We hypothesized that alternatively activated M2 macrophages are abnormal in CF lung disease. METHODS: Blood samples were collected from adults (n=13) children (n=27) with CF on admission for acute pulmonary exacerbation and when clinically stable. Monocytes were differentiated into macrophages and polarized into classical (M1) and alternatively-activated (M2) phenotypes, function determined ex-vivo and compared with healthy controls...
April 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28427956/curcumin-poly-2-methyl-2-oxazoline-b-tetrahydrofuran-b-2-methyl-2-oxazoline-formulation-an-improved-penetration-and-biological-effect-of-curcumin-in-f508del-cftr-cell-lines
#4
Cristine Gonçalves, Jean-Pierre Gomez, William Même, Bazoly Rasolonjatovo, David Gosset, Steven Nedellec, Philippe Hulin, Cécile Huin, Tony Le Gall, Tristan Montier, Pierre Lehn, Chantal Pichon, Philippe Guégan, Hervé Cheradame, Patrick Midoux
Neutral amphiphilic triblock ABA copolymers are of great interest to solubilize hydrophobic drugs. We reported that a triblock ABA copolymer consisting of methyl-2-oxazoline (MeOx) and tetrahydrofuran (THF) (MeOx6-THF19-MeOx6) (TBCP2) can solubilize curcumin (Cur) a very hydrophobic molecule exhibiting multiple therapeutic effects but whose insolubility and low stability in water is a major drawback for clinical applications. Here, we provide evidences by flow cytometry and confocal microscopy that Cur penetration in normal and ΔF508-CFTR human airway epithelial cell lines is facilitated by TBCP2...
April 17, 2017: European Journal of Pharmaceutics and Biopharmaceutics
https://www.readbyqxmd.com/read/28424169/identification-of-the-fatty-acid-activation-site-on-human-clc-2
#5
John Cuppoletti, Kirti P Tewari, Jayati Chakrabarti, Danuta H Malinowska
Fatty acids (including lubiprostone and cobiprostone) are human ClC-2 (hClC-2) Cl(-) channel activators. Molecular and cellular mechanisms underlying this activation were examined. Role of a 4-amino acid PKA activation site, RGET691 of hClC-2 was investigated using WT and mutant (AGET, RGEA and AGAA) hClC-2 expressed in 293EBNA cells as well as involvement of PKA, [cAMP]i, EP2 or EP4 receptor agonist activity. All fatty acids (lubiprostone, cobiprostone, eicosatetraynoic acid (ETYA), oleic acid and elaidic acid) caused significant rightward shifts in concentration-dependent Cl(-) current activation (increasing EC50s) with mutant compared to WT hClC-2 channels, without changing time- and voltage-dependence, I-V rectification or methadone inhibition of the channel...
April 19, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28419121/the-magnitude-of-ivacaftor-effects-on-fluid-secretion-via-r117h-cftr-channels-human-in-vivo-measurements
#6
Jessica E Char, Colleen Dunn, Zoe Davies, Carlos Milla, Richard B Moss, Jeffrey J Wine
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. We injected β-adrenergic agonists intradermally to stimulate CFTR-dependent 'C-sweat' and methacholine to stimulate 'M-sweat', which persists in CF subjects. We focused on an R117H-7T/F508del subject who produced quantifiable C-sweat off ivacaftor and was available for 1 blinded, 3 off ivacaftor, and 3 on ivacaftor tests, allowing us to estimate in vivo fold-increase in sweat rates produced by ivacaftor's effect on the open probability (PO) of R117H-CFTR...
2017: PloS One
https://www.readbyqxmd.com/read/28415617/upregulation-of-cftr-in-patients-with-endometriosis-and-its-involvement-in-nf%C3%AE%C2%BAb-upar-dependent-cell-migration
#7
Wenqing Huang, Aihong Jin, Jieting Zhang, Chaoqun Wang, Lai Ling Tsang, Zhiming Cai, Xiaping Zhou, Hao Chen, Hsiao Chang Chan
Endometriotic tissues exhibit high migration ability with the underlying mechanisms remain elusive. Our previous studies have demonstrated that cystic fibrosis transmembrane conductance regulator (CFTR) acts as a tumor suppressor regulating cell migration. In the present study, we explored whether CFTR plays a role in the development of human endometriosis. We found that both mRNA and protein expression levels of CFTR and urokinase-type plasminogen activator receptor (uPAR) were significantly increased in ectopic endometrial tissues from patients with endometriosis compared to normal endometrial tissues from women without endometriosis and positively correlated...
March 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28408918/cftr-gene-mutations-in-the-egyptian-population-current-and-future-insights-for-genetic-screening-strategy
#8
Ayman S El-Seedy, Hanaa Shafiek, Alain Kitzis, Véronique Ladevèze
No abstract text is available yet for this article.
2017: Frontiers in Genetics
https://www.readbyqxmd.com/read/28407399/abnormal-rab11-rab8-vesicles-cluster-in-enterocytes-of-patients-with-microvillus-inclusion-disease
#9
Georg F Vogel, Andreas R Janecke, Iris M Krainer, Karin Gutleben, Barbara Witting, Sally G Mitton, Sahar Mansour, Antje Ballauff, Joseph T Roland, Amy C Engevik, Ernest Cutz, Thomas Müller, James R Goldenring, Lukas A Huber, Michael W Hess
Microvillus Inclusion Disease (MVID) is a congenital enteropathy characterized by accumulation of vesiculo-tubular endomembranes in the subapical cytoplasm of enterocytes, historically termed "secretory granules". However, neither their identity nor pathophysiological significance is well defined. Using immunoelectron microscopy and tomography we studied biopsies from MVID patients (3x Myosin 5b mutations, 1x Syntaxin3 mutation) and compared them to controls and genome-edited CaCo2 cell models, harboring relevant mutations...
April 13, 2017: Traffic
https://www.readbyqxmd.com/read/28406713/an-observational-study-of-outcomes-and-tolerances-in-patients-with-cystic-fibrosis-initiated-on-lumacaftor-ivacaftor
#10
Mark T Jennings, Rebecca Dezube, Shruti Paranjape, Natalie E West, Gina Hong, Andrew Braun, Jonathan Grant, Christian A Merlo, Noah Lechtzin
RATIONALE: In July 2015, the FDA approved lumacaftor/ivacaftor for use in patients with CF. This drug targets the primary defect in the CFTR protein that is conferred by the F508del CFTR mutation. OBJECTIVE: As there is limited experience with this therapy outside of clinical trials, this study aims to examine the clinical experience of this new drug in a CF population. METHODS: Retrospective cohort study of individuals followed at the Johns Hopkins CF Center, who initiated treatment with lumacaftor/ivacaftor...
April 13, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28394330/thymosin-%C3%AE-1-represents-a-potential-potent-single-molecule-based-therapy-for-cystic-fibrosis
#11
Luigina Romani, Vasilis Oikonomou, Silvia Moretti, Rossana G Iannitti, Maria Cristina D'Adamo, Valeria R Villella, Marilena Pariano, Luigi Sforna, Monica Borghi, Marina M Bellet, Francesca Fallarino, Maria Teresa Pallotta, Giuseppe Servillo, Eleonora Ferrari, Paolo Puccetti, Guido Kroemer, Mauro Pessia, Luigi Maiuri, Allan L Goldstein, Enrico Garaci
Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) that compromise its chloride channel activity. The most common mutation, p.Phe508del, results in the production of a misfolded CFTR protein, which has residual channel activity but is prematurely degraded. Because of the inherent complexity of the pathogenetic mechanisms involved in CF, which include impaired chloride permeability and persistent lung inflammation, a multidrug approach is required for efficacious CF therapy...
April 10, 2017: Nature Medicine
https://www.readbyqxmd.com/read/28392259/active-site-flexibility-and-substrate-specificity-in-a-bacterial-virulence-factor-crystallographic-snapshots-of-an-epoxide-hydrolase
#12
Kelli L Hvorecny, Christopher D Bahl, Seiya Kitamura, Kin Sing Stephen Lee, Bruce D Hammock, Christophe Morisseau, Dean R Madden
Pseudomonas aeruginosa secretes an epoxide hydrolase with catalytic activity that triggers degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and perturbs other host defense networks. Targets of this CFTR inhibitory factor (Cif) are largely unknown, but include an epoxy-fatty acid. In this class of signaling molecules, chirality can be an important determinant of physiological output and potency. Here we explore the active-site chemistry of this two-step α/β-hydrolase and its implications for an emerging class of virulence enzymes...
March 31, 2017: Structure
https://www.readbyqxmd.com/read/28392015/modeling-cystic-fibrosis-disease-progression-in-patients-with-the-rare-cftr-mutation-p67l
#13
Isobel E R MacKenzie, Valerie Paquette, Frances Gosse, Sheenagh George, Frederic Chappe, Valerie Chappe
BACKGROUND: The progression of cystic fibrosis (CF) in patients with the rare mutation P67L was examined to determine if it induced a milder form of CF compared to the common severe ΔF508 mutation. METHODS: Parameters of lung function, level of bacterial infection, nutritional status and hospitalization were used to represent CF progression. Age at diagnosis and pancreatic status were used to assess CF presentation. Analysis of data from the CF Canada Registry collected over a 15-year period included 266 ΔF508/ΔF508 homozygote patients from CF clinics in Atlantic Canada and 26 compound heterozygote patients with the rare P67L mutation from clinics across Canada...
April 5, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28384265/growth-deficits-in-cystic-fibrosis-mice-begin-in-utero-prior-to-igf-1-reduction
#14
Rebecca Darrah, Ilya Bederman, Megan Vitko, Dana M Valerio, Mitchell L Drumm, Craig A Hodges
Growth deficits are common in cystic fibrosis (CF), but their cause is complex, with contributions from exocrine pancreatic insufficiency, pulmonary complications, gastrointestinal obstructions, and endocrine abnormalities. The CF mouse model displays similar growth impairment despite exocrine pancreatic function and in the absence of chronic pulmonary infection. The high incidence of intestinal obstruction in the CF mouse has been suggested to significantly contribute to the observed growth deficits. Previous studies by our group have shown that restoration of the cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium prevents intestinal obstruction but does not improve growth...
2017: PloS One
https://www.readbyqxmd.com/read/28384194/loss-of-slc9a3-decreases-cftr-protein-and-causes-obstructed-azoospermia-in-mice
#15
Ya-Yun Wang, Ying-Hung Lin, Yi-No Wu, Yen-Lin Chen, Yung-Chih Lin, Chiao-Yin Cheng, Han-Sun Chiang
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF) and are associated with congenital bilateral absence of the vas deferens (CBAVD), which is the major cause of infertility in male patients with CF. However, most Taiwanese patients with CBAVD do not carry major CFTR mutations. Some patients have a single copy deletion of the solute carrier family 9 isoform 3 (SLC9A3) gene. SLC9A3 is a Na+/H+ exchanger, and depleted Slc9a3 in male mice causes infertility due to the abnormal dilated lumen of the rete testis and efferent ductules...
April 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28378305/water-saving-technologies-affect-the-grain-characteristics-and-recovery-of-fine-grain-rice-cultivars-in-semi-arid-environment
#16
Khawar Jabran, Muhammad Riaz, Mubshar Hussain, Wajid Nasim, Umar Zaman, Shah Fahad, Bhagirath Singh Chauhan
Growing rice with less water is direly needed due to declining water sources worldwide, but using methods that require less water inputs can have an impact on grain characteristics and recovery. A 2-year field study was conducted to evaluate the impact of conventionally sown flooded rice and low-water-input rice systems on the grain characteristics and recovery of fine rice. Three fine grain rice cultivars-Super Basmati, Basmati 2000, and Shaheen Basmati-were grown under conventional flooded transplanted rice (CFTR), alternate wetting and drying (AWD), and aerobic rice systems...
April 4, 2017: Environmental Science and Pollution Research International
https://www.readbyqxmd.com/read/28377087/monocyte-derived-macrophages-from-cf-pigs-exhibit-increased-inflammatory-responses-at-birth
#17
Lily Paemka, Brian N McCullagh, Mahmoud H Abou Alaiwa, David A Stoltz, Qian Dong, Christoph O Randak, Robert D Gray, Paul B McCray
BACKGROUND: We sought to address whether CF macrophages have a primary functional defect as a consequence of CFTR loss and thus contribute to the onset of infection and inflammation observed in CF lung disease. METHODS: Monocyte derived macrophages (MDMs) were prepared from newborn CF and non-CF pigs. CFTR mRNA expression was quantified by rtPCR and anion channel function was determined using whole cell patch clamp analysis. IL8 and TNFα release from MDMs in response to lipopolysaccharide stimulation was measured by ELISA...
April 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28376702/high-throughput-screening-for-readthrough-modulators-of-cftr-ptc-mutations
#18
Feng Liang, Haibo Shang, Nikole J Jordan, Eric Wong, Dayna Mercadante, Josef Saltz, Jerome Mahiou, Hermann J Bihler, Martin Mense
Cystic fibrosis (CF) is a hereditary disease caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR). A large number of nearly 2000 reported mutations, including the premature termination codon (PTC) mutations, urgently require new and personalized medicines. We have developed cell-based assays for readthrough modulators of CFTR PTC mutations (or nonsense mutation suppressors), based on the trafficking and surface expression of CFTR. Approximately 85,000 compounds have been screened for two PTC mutations (Y122X and W1282X)...
February 1, 2017: SLAS Technology
https://www.readbyqxmd.com/read/28371569/pseudomonas-eradication-and-clinical-effectivness-of-ivacaftor-in-four-hispanic-patients-with-s549n
#19
Abigail Strang, Anthony J Fischer, Aaron Chidekel
Ivacaftor was approved for rarer class-III CFTR mutations including S549N in 2014. Since these mutations are uncommon, ongoing reports of patient experiences with Ivacaftor and these mutations are important. This case series describes the clinical effectiveness (including airway infection status, lung function, and growth) of Ivacaftor therapy in four pediatric Hispanic patients with S549N and F508del over 24 months. In these patients, Ivacaftor was highly efficacious with no further Pseudomonas-positive cultures despite prior chronic colonization in three patients as well as notable improvements in lung function and growth...
April 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28366727/corrector-vx-809-promotes-interactions-between-cytoplasmic-loop-one-and-the-first-nucleotide-binding-domain-of-cftr
#20
Tip W Loo, David M Clarke
A large number of correctors have been identified that can partially repair defects in folding, stability and trafficking of CFTR processing mutants that cause cystic fibrosis (CF). The best corrector, VX-809 (Lumacaftor), has shown some promise when used in combination with a potentiator (Ivacaftor). Understanding the mechanism of VX-809 is essential for development of better correctors. Here, we tested our prediction that VX-809 repairs folding and processing defects of CFTR by promoting interactions between the first cytoplasmic loop (CL1) of transmembrane domain 1 (TMD1) and the first nucleotide-binding domain (NBD1)...
March 30, 2017: Biochemical Pharmacology
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