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https://www.readbyqxmd.com/read/28230981/high-potency-phenylquinoxalinone-cystic-fibrosis-transmembrane-conductance-regulator-cftr-activators
#1
Jung-Ho Son, Jie S Zhu, Puay-Wah Phuan, Onur Cil, Andrew P Teuthorn, Colton K Ku, Sujin Lee, Alan S Verkman, Mark J Kurth
We previously identified phenylquinoxalinone CFTRact-J027 (4) as a cystic fibrosis transmembrane conductance regulator (CFTR) activator with an EC50 of ~200 nM, and demonstrated its therapeutic efficacy in mouse models of constipation. Here, structure-activity studies were done on 36 synthesized phenylquinoxalinone analogs to identify compounds with improved potency and altered metabolic stability. Synthesis of the phenylquinoxalinone core was generally accomplished by condensation of 1,2-phenylenediamines with substituted phenyloxoacetates...
February 23, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28230279/folate-protects-hepatocytes-of-hyperhomocysteinemia-mice-from-apoptosis-via-cystic-fibrosis-transmembrane-conductance-regulator-cftr-activated-endoplasmic-reticulum-stress
#2
Anning Yang, Yue Sun, Caiyan Mao, Songhao Yang, Min Huang, Mei Deng, Ning Ding, Xiaoling Yang, Minghao Zhang, Shaoju Jin, Yideng Jiang, Ying Huang
Folate deficiency is a known risk factor for liver injury; however, the underlying mechanism remains unclear. In this study, we employed a high homocysteine-induced liver injury model of Apolipoprotein E-deficient (ApoE(-/-) ) mice fed high-methionine diet and found that high homocysteine induced endoplasmic reticulum (ER) stress and liver cell apoptosis by downregulation of cystic fibrosis transmembrane conductance regulator (CFTR) expression; observations that were attenuated with supplementation of dietary folate...
February 23, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28225751/lower-cystic-fibrosis-transmembrane-conductance-regulator-cftr-promotes-the-proliferation-and-migration-of-endometrial-carcinoma
#3
Xian Xia, Jie Wang, Yuan Liu, Ming Yue
BACKGROUND The incidence and death rates of endometrial cancer are alarmingly increasing. The diagnosis and treatment of endometrial cancer is crucial to decreasing mortality. Cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the adenosine triphosphate (ATP)-binding cassette transporter family and plays an essential role in anion regulation and tissue homeostasis of various epithelia. This study explored the expression of CFTR in endometrial carcinoma and the role of CFTR in proliferation and migration of endometrial carcinoma cells...
February 22, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28222269/restoring-cftr-function-reduces-airway-bacteria-and-inflammation-in-people-with-cystic-fibrosis-and-chronic-lung-infections
#4
Katherine B Hisert, Sonya L Heltshe, Christopher Pope, Peter Jorth, Xia Wu, Rachael M Edwards, Matthew Radey, Frank J Accurso, Daniel J Wolter, Gordon Cooke, Ryan J Adam, Suzanne Carter, Brenda Grogan, Jan L Launspach, Seamas C Donnelly, Charles Gallagher, James E Bruce, David Stoltz, Michael J Welsh, Lucas R Hoffman, Edward F McKone, Pradeep K Singh
RATIONALE: Previous work indicates that ivacaftor improves CFTR activity and lung function in people with cystic fibrosis (CF) and G551D-CFTR mutations, but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once CF lung disease is established. OBJECTIVES: To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with CF and chronic airway infections...
February 21, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28221098/ion-transport-by-ameloblasts-during-amelogenesis
#5
A L J J Bronckers
Hypomineralization of developing enamel is associated with changes in ameloblast modulation during the maturation stage. Modulation (or pH cycling) involves the cyclic transformation of ruffle-ended (RE) ameloblasts facing slightly acidic enamel into smooth-ended (SE) ameloblasts near pH-neutral enamel. The mechanism of ameloblast modulation is not clear. Failure of ameloblasts of Cftr-null and anion exchanger 2 ( Ae2)-null mice to transport Cl(-) into enamel acidifies enamel, prevents modulation, and reduces mineralization...
March 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/28215711/biomarkers-for-cystic-fibrosis-drug-development
#6
REVIEW
Marianne S Muhlebach, J P Clancy, Sonya L Heltshe, Assem Ziady, Tom Kelley, Frank Accurso, Joseph Pilewski, Nicole Mayer-Hamblett, Elizabeth Joseloff, Scott D Sagel
PURPOSE: To provide a review of the status of biomarkers in cystic fibrosis drug development, including regulatory definitions and considerations, a summary of biomarkers in current use with supportive data, current gaps, and future needs. METHODS: Biomarkers are considered across several areas of CF drug development, including cystic fibrosis transmembrane conductance regulator modulation, infection, and inflammation. RESULTS: Sweat chloride, nasal potential difference, and intestinal current measurements have been standardized and examined in the context of multicenter trials to quantify CFTR function...
November 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28213469/insulin-signaling-via-the-pi3k-akt-pathway-regulates-airway-glucose-uptake-and-barrier-function-in-a-cftr-dependent-manner
#7
Samuel A Molina, Hannah K Moriarty, Danny T Infield, Barry R Imhoff, Rachel J Vance, Agnes H Kim, Jason M Hansen, William R Hunt, Michael Koval, Nael A McCarty
Cystic fibrosis-related diabetes (CFRD) is the most common co-morbidity associated with cystic fibrosis (CF) and correlates with increased rates of lung function decline. Since glucose is a nutrient present in the airways of patients with bacterial airway infections and since insulin controls glucose metabolism, the effect of insulin on CF airway epithelia was investigated to determine the role of insulin receptors and glucose transport in regulating glucose availability in the airway. The response to insulin by human airway epithelial cells was characterized by qPCR, immunoblot, immunofluorescence, and glucose uptake assays...
February 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28209466/pharmacokinetics-and-safety-of-cavosonstat-n91115-in-healthy-and-cystic-fibrosis-adults-homozygous-for-f508del-cftr
#8
Scott H Donaldson, George M Solomon, Pamela L Zeitlin, Patrick A Flume, Alicia Casey, Karen McCoy, Edith T Zemanick, Arun Mandagere, Janice M Troha, Steven A Shoemaker, James F Chmiel, Jennifer L Taylor-Cousar
BACKGROUND: Cavosonstat (N91115), an orally bioavailable inhibitor of S-nitrosoglutathione reductase, promotes cystic fibrosis transmembrane conductance regulator (CFTR) maturation and plasma membrane stability, with a mechanism of action complementary to CFTR correctors and potentiators. METHODS: A Phase I program evaluated pharmacokinetics, drug-drug interactions and safety of cavosonstat in healthy and cystic fibrosis (CF) subjects homozygous for F508del-CFTR...
February 13, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28209107/gastro-intestinal-manifestations-in-cystic-fibrosis-patients
#9
REVIEW
S Van Biervliet, C de Clercq, D Declercq, E Van Braeckel, S Van Daele, F De Baets, D De Looze
Cystic fibrosis (CF) is a life-limiting disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). This defective chloride channel, present in different organ systems such as respiratory system, gastrointestinal tract, reproductive system and sweat glands, disturbs the ion and water transport over the membranes leading to the well known CF symptoms. CF has outgrown paediatric care, as half of CF patients are currently adults. The CF gastrointestinal tract has its own particularities...
September 2016: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/28208841/assessment-of-correlation-between-sweat-chloride-levels-and-clinical-features-of-cystic-fibrosis-patients
#10
Manzoor A Raina, Mosin S Khan, Showkat A Malik, Ab Hameed Raina, Mudassir J Makhdoomi, Javed I Bhat, Syed Mudassar
INTRODUCTION: Cystic Fibrosis (CF) is an autosomal recessive disorder and the incidence of this disease is undermined in Northern India. The distinguishable salty character of the sweat belonging to individuals suffering from CF makes sweat chloride estimation essential for diagnosis of CF disease. AIM: The aim of this prospective study was to elucidate the relationship of sweat chloride levels with clinical features and pattern of CF. MATERIALS AND METHODS: A total of 182 patients, with clinical features of CF were included in this study for quantitative measurement of sweat chloride...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28204942/molecular-modeling-in-the-age-of-clinical-genomics-the-enterprise-of-the-next-generation
#11
Jeremy W Prokop, Jozef Lazar, Gabrielle Crapitto, D Casey Smith, Elizabeth A Worthey, Howard J Jacob
Protein modeling and molecular dynamics hold a unique toolset to aide in the characterization of clinical variants that may result in disease. Not only do these techniques offer the ability to study under characterized proteins, but they do this with the speed that is needed for time-sensitive clinical cases. In this paper we retrospectively study a clinical variant in the XIAP protein, C203Y, while addressing additional variants seen in patients with similar gastrointestinal phenotypes as the C203Y mutation...
March 2017: Journal of Molecular Modeling
https://www.readbyqxmd.com/read/28203405/weighted-gene-co-expression-network-analysis-in-identification-of-metastasis-related-genes-of-lung-squamous-cell-carcinoma-based-on-the-cancer-genome-atlas-database
#12
Feng Tian, Jinlong Zhao, Xinlei Fan, Zhenxing Kang
BACKGROUND: Lung squamous cell carcinoma (lung SCC) is a common type of malignancy. Its pathogenesis mechanism of tumor development is unclear. The aim of this study was to identify key genes for diagnosis biomarkers in lung SCC metastasis. METHODS: We searched and downloaded mRNA expression data and clinical data from The Cancer Genome Atlas (TCGA) database to identify differences in mRNA expression of primary tumor tissues from lung SCC with and without metastasis...
January 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28198020/beyond-cystic-fibrosis-transmembrane-conductance-regulator-cftr-single-channel-kinetics-implications-for-therapeutic-intervention
#13
Carmel M McNicholas
No abstract text is available yet for this article.
February 15, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28196856/vardenafil-reduces-macrophage-pro-inflammatory-overresponses-in-cystic-fibrosis-through-pde5-and-cftr-dependent-mechanisms
#14
Sabrina Noel, Nadtha Panin, Mathilde Beka, Barbara Dhooghe, Francois Huaux, Teresinha Leal
Chronic inflammation that progressively disrupts the lung tissue is a hallmark of Cystic Fibrosis (CF). In mice, vardenafil, an inhibitor of phosphodiesterase type 5 (PDE5), restores transepithelial ion transport and corrects mislocalization of the most common CF mutation, F508del-CFTR. It also reduces lung pro-inflammatory responses in mice and in patients with CF. To test the hypothesis that macrophages are target effector cells of the immunomodulatory effect of vardenafil, we isolated lung macrophages from mice homozygous for the F508del mutation or invalidated for the cftr gene and from their corresponding wild-type littermates...
February 14, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28196530/r248g-cystic-fibrosis-transmembrane-conductance-regulator-mutation-in-three-siblings-presenting-with-recurrent-acute-pancreatitis-and-reproductive-issues-a-case-series
#15
Seiichi Villalona, Guillermo Glover-López, Juan Antonio Ortega-García, Rosa Moya-Quiles, Pedro Mondejar-López, Maria C Martínez-Romero, Mariano Rigabert-Montiel, María D Pastor-Vivero, Manuel Sánchez-Solís
BACKGROUND: Mutational combinations of the cystic fibrosis transmembrane conductance regulator, CFTR, gene have different phenotypic manifestations at the molecular level with varying clinical consequences for individuals possessing such mutations. Reporting cystic fibrosis transmembrane conductance regulator mutations is important in understanding the genotype-phenotype correlations and associated clinical presentations in patients with cystic fibrosis. Understanding the effects of mutations is critical in developing appropriate treatments for individuals affected with cystic fibrosis, non-classic cystic fibrosis, or cystic fibrosis transmembrane conductance regulator-related disorders...
February 15, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28194692/role-of-cftr-mutation-analysis-in-the-diagnostic-algorithm-for-cystic-fibrosis
#16
Michelle Ratkiewicz, Matthew Pastore, Karen Sharrock McCoy, Rohan Thompson, Don Hayes, Shahid Ijaz Sheikh
BACKGROUND: The cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation identification is being used with increased frequency to aid in the diagnosis of cystic fibrosis (CF) in those suspected with CF. Aim of this study was to identify diagnostic outcomes when CFTR mutational analysis was used in CF diagnosis. CFTR mutational analysis results were also compared with sweat chloride results. METHODS: This study was done on all patients at our institution who had CFTR mutation analysis over a sevenyear period since August 2006...
February 14, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28192405/cftr-is-a-tumor-suppressor-gene-in-murine-and-human-intestinal-cancer
#17
B L N Than, J F Linnekamp, T K Starr, D A Largaespada, A Rod, Y Zhang, V Bruner, J Abrahante, A Schumann, T Luczak, J Walter, A Niemczyk, M G O'Sullivan, J P Medema, R J A Fijneman, G A Meijer, E Van den Broek, C A Hodges, P M Scott, L Vermeulen, R T Cormier
No abstract text is available yet for this article.
February 13, 2017: Oncogene
https://www.readbyqxmd.com/read/28190780/pregnancy-among-cystic-fibrosis-women-in-the-era-of-cftr-modulators
#18
Sonya L Heltshe, Emily M Godfrey, Tatiana Josephy, Moira L Aitken, Jennifer L Taylor-Cousar
BACKGROUND: Little is known about how new therapies that partially correct the basic cystic fibrosis (CF) defect (ivacaftor and lumacaftor) might alter hormonal contraceptive effectiveness, impact pregnancy outcomes, or affect pregnancy timing. Examination of pregnancy rates among CF women during periods of CFTR modulator therapy initiation will provide foundation for further research in this area. METHODS: The Cystic Fibrosis Foundation Patient Registry was used to examine pregnancy rates and outcomes by genotype class before, during, and after the introduction of CFTR modulator therapies between 2005 and 2014...
February 9, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28187947/partial-restoration-of-cftr-function-in-cftr-null-mice-following-targeted-cell-replacement-therapy
#19
Pascal Duchesneau, Rickvinder Besla, Mathieu F Derouet, Li Guo, Golnaz Karoubi, Amanda Silberberg, Amy P Wong, Thomas K Waddell
Cystic fibrosis (CF) is a fatal recessive genetic disorder caused by a mutation in the gene encoding CF transmembrane conductance regulator (CFTR) protein. Alteration in CFTR leads to thick airway mucus and bacterial infection. Cell therapy has been proposed for CFTR restoration, but efficacy has been limited by low engraftment levels. In our previous studies, we have shown that using a pre-conditioning regimen in combination with optimization of cell number and time of delivery, we could obtain greater bone marrow cell (BMC) retention in the lung...
February 8, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28185838/vitamin-k-status-in-cystic-fibrosis-patients-with-liver-cirrhosis
#20
Patrycja Krzyżanowska, Sławomira Drzymała-Czyż, Andrzej Pogorzelski, Monika Duś-Żuchowska, Wojciech Skorupa, Lyudmyla Bober, Ewa Sapiejka, Beata Oralewska, Nataliya Rohovyk, Jerzy Moczko, Jan Nowak, Ewa Wenska-Chyży, Marta Rachel, Aleksandra Lisowska, Jarosław Walkowiak
The available data on the influence of liver cirrhosis on vitamin K status in CF patients is scarce. Therefore, the aims of the present study were to assess the prevalence of vitamin K deficiency in cirrhotic CF subjects and to determine whether it correlates with liver cirrhosis. The study group comprised of 27 CF patients with and 63 without liver cirrhosis. Vitamin K status was assessed using prothrombin induced by vitamin K absence (PIVKA-II) and the percentage of undercarboxylated osteocalcin (u-OC). PIVKA-II concentrations were higher in cirrhotic than in non-cirrhotic CF patients (median [1st-3rd quartile]: 3...
January 23, 2017: Digestive and Liver Disease
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