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Journal articles on the topic 'Cystic fibrosis transmembrane'

Author: Grafiati

Published: 4 June 2021

Last updated: 12 February 2022

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1

Smith, Stephen S., Erich D. Steinle, Mark E. Meyerhoff, and David C. Dawson. "Cystic Fibrosis Transmembrane Conductance Regulator." Journal of General Physiology 114, no. 6 (November 29, 1999): 799–818. http://dx.doi.org/10.1085/jgp.114.6.799.

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The cystic fibrosis transmembrane conductance regulator (CFTR) Cl channel exhibits lyotropic anion selectivity. Anions that are more readily dehydrated than Cl exhibit permeability ratios (PS/PCl) greater than unity and also bind more tightly in the channel. We compared the selectivity of CFTR to that of a synthetic anion-selective membrane [poly(vinyl chloride)–tridodecylmethylammonium chloride; PVC-TDMAC] for which the nature of the physical process that governs the anion-selective response is more readily apparent. The permeability and binding selectivity patterns of CFTR differed only by a multiplicative constant from that of the PVC-TDMAC membrane; and a continuum electrostatic model suggested that both patterns could be understood in terms of the differences in the relative stabilization of anions by water and the polarizable interior of the channel or synthetic membrane. The calculated energies of anion–channel interaction, derived from measurements of either permeability or binding, varied as a linear function of inverse ionic radius (1/r), as expected from a Born-type model of ion charging in a medium characterized by an effective dielectric constant of 19. The model predicts that large anions, like SCN, although they experience weaker interactions (relative to Cl) with water and also with the channel, are more permeant than Cl because anion–water energy is a steeper function of 1/r than is the anion–channel energy. These large anions also bind more tightly for the same reason: the reduced energy of hydration allows the net transfer energy (the well depth) to be more negative. This simple selectivity mechanism that governs permeability and binding acts to optimize the function of CFTR as a Cl filter. Anions that are smaller (more difficult to dehydrate) than Cl are energetically retarded from entering the channel, while the larger (more readily dehydrated) anions are retarded in their passage by “sticking” within the channel.

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2

Akabas, Myles H. "Cystic Fibrosis Transmembrane Conductance Regulator." Journal of Biological Chemistry 275, no. 6 (February 11, 2000): 3729–32. http://dx.doi.org/10.1074/jbc.275.6.3729.

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3

Antoniu, Sabina Antonela. "Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies." Expert Opinion on Investigational Drugs 20, no. 3 (February 9, 2011): 423–25. http://dx.doi.org/10.1517/13543784.2011.554823.

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4

Noel, Sabrina, Bela Z. Schmidt, Jeremy Haaf, and Teresinha Leal. "Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives." Clinical Pharmacology: Advances and Applications Volume 8 (September 2016): 127–40. http://dx.doi.org/10.2147/cpaa.s100759.

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5

MacDonald, Kelvin D., Karen R. McKenzie, and Pamela L. Zeitlin. "Cystic Fibrosis Transmembrane Regulator Protein Mutations." Pediatric Drugs 9, no. 1 (2007): 1–10. http://dx.doi.org/10.2165/00148581-200709010-00001.

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6

Dawson, David C., and Stephen S. Smith. "Commentary Cystic Fibrosis Transmembrane Conductance Regulator." Journal of General Physiology 110, no. 4 (October 1, 1997): 337–39. http://dx.doi.org/10.1085/jgp.110.4.337.

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7

Zemanick, Edith T., and Frank J. Accurso. "Cystic Fibrosis Transmembrane Conductance Regulator andPseudomonas." American Journal of Respiratory and Critical Care Medicine 189, no. 7 (April 2014): 763–65. http://dx.doi.org/10.1164/rccm.201402-0209ed.

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8

Riordan, J. R. "The Cystic Fibrosis Transmembrane Conductance Regulator." Annual Review of Physiology 55, no. 1 (October 1993): 609–30. http://dx.doi.org/10.1146/annurev.ph.55.030193.003141.

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9

Burgener, Elizabeth B., and Richard B. Moss. "Cystic fibrosis transmembrane conductance regulator modulators." Current Opinion in Pediatrics 30, no. 3 (June 2018): 372–77. http://dx.doi.org/10.1097/mop.0000000000000627.

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10

Corradi, Valentina, Paola Vergani, and D. Peter Tieleman. "Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)." Journal of Biological Chemistry 290, no. 38 (July 30, 2015): 22891–906. http://dx.doi.org/10.1074/jbc.m115.665125.

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11

Higgins, C. F. "Cystic fibrosis transmembrane conductance regulator (CFTR)." British Medical Bulletin 48, no. 4 (1992): 754–65. http://dx.doi.org/10.1093/oxfordjournals.bmb.a072576.

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12

Bingol, A., M. G. Ertosun, R. Artan, A. Yilmaz, E. Mihci, B. N. Guzel, M. Erman Akar, et al. "35 Cystic fibrosis transmembrane regulator mutations in Turkish patients with cystic fibrosis." Journal of Cystic Fibrosis 13 (June 2014): S55. http://dx.doi.org/10.1016/s1569-1993(14)60172-7.

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13

Marcet, Brice, and Jean-Marie Boeynaems. "Relationships between cystic fibrosis transmembrane conductance regulator, extracellular nucleotides and cystic fibrosis." Pharmacology & Therapeutics 112, no. 3 (December 2006): 719–32. http://dx.doi.org/10.1016/j.pharmthera.2006.05.010.

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14

Egan, Marie E. "Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update." Current Opinion in Pediatrics 32, no. 3 (June 2020): 384–88. http://dx.doi.org/10.1097/mop.0000000000000892.

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15

Bienvenu, Thierry, Isabelle Sermet-Gaudelus, Pierre-Regis Burgel, Dominique Hubert, Bruno Crestani, Laurence Bassinet, Daniel Dusser, and Isabelle Fajac. "Cystic Fibrosis Transmembrane Conductance Regulator Channel Dysfunction in Non–Cystic Fibrosis Bronchiectasis." American Journal of Respiratory and Critical Care Medicine 181, no. 10 (May 15, 2010): 1078–84. http://dx.doi.org/10.1164/rccm.200909-1434oc.

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16

Mekus, Frauke, Manfred Ballmann, Inez Bronsveld, Thilo Dörk, Jan Bijman, Burkhard Tümmler, and H. J. Veeze. "Cystic-fibrosis-like disease unrelated to the cystic fibrosis transmembrane conductance regulator." Human Genetics 102, no. 5 (May 29, 1998): 582–86. http://dx.doi.org/10.1007/s004390050744.

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17

Furgeri, Daniela Tenório, Fernando Augusto Lima Marson, Cyntia Arivabeni Araújo Correia, José Dirceu Ribeiro, and Carmen Sílvia Bertuzzo. "Cystic fibrosis transmembrane regulator haplotypes in households of patients with cystic fibrosis." Gene 641 (January 2018): 137–43. http://dx.doi.org/10.1016/j.gene.2017.10.052.

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18

Staab, D. "Cystic fibrosis -- therapeutic challenge in cystic fibrosis children." European Journal of Endocrinology 151, Suppl_1 (August 1, 2004): S77—S80. http://dx.doi.org/10.1530/eje.0.151s077.

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Cystic fibrosis (CF) is the most common autosomal recessive disease with fatal outcome in Caucasians with a frequency of 1 in 2500 life births. It is caused by mutations in a single gene on the long arm of chromosome 7 encoding a protein called the cystic fibrosis transmembrane regulator (CFTR). The defect in CFTR leads to pathological changes in all organs with mucus-secretory glands, e.g. airways, pancreas, gut, biliary tract, vas deferens and sweat glands. Despite impressive advances in understanding the molecular basis of the disease, life expectancy is still limited in CF and chronic infection of the lung resulting in fibrosis and bronchiectasis followed by respiratory insufficiency is still the main factor in morbidity and the leading cause of death. Poor nutritional status is one of the major problems in the vicious cycle of chronic inflammation and lung destruction and its impact on outcome in lung function has been demonstrated. The possible role of growth hormone treatment in this context will be discussed.

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19

Banks, Catherine, Laura Freeman, Do Yeon Cho, and Bradford A. Woodworth. "Acquired cystic fibrosis transmembrane conductance regulator dysfunction." World Journal of Otorhinolaryngology - Head and Neck Surgery 4, no. 3 (September 2018): 193–99. http://dx.doi.org/10.1016/j.wjorl.2018.09.001.

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20

Hunt, J. F., C. Wang, and R. C. Ford. "Cystic Fibrosis Transmembrane Conductance Regulator (ABCC7) Structure." Cold Spring Harbor Perspectives in Medicine 3, no. 2 (February 1, 2013): a009514. http://dx.doi.org/10.1101/cshperspect.a009514.

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21

Rowe, S. M., and A. S. Verkman. "Cystic Fibrosis Transmembrane Regulator Correctors and Potentiators." Cold Spring Harbor Perspectives in Medicine 3, no. 7 (July 1, 2013): a009761. http://dx.doi.org/10.1101/cshperspect.a009761.

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22

Tsui, Lap-Chee. "The Cystic Fibrosis Transmembrane Conductance Regulator Gene." American Journal of Respiratory and Critical Care Medicine 151, no. 3_pt_2 (March 1995): S47—S53. http://dx.doi.org/10.1164/ajrccm/151.3_pt_2.s47.

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23

Fuller, C. M., M. B. Howard, D. M. Bedwell, R. A. Frizzell, and D. J. Benos. "Antibodies against the cystic fibrosis transmembrane regulator." American Journal of Physiology-Cell Physiology 262, no. 2 (February 1, 1992): C396—C404. http://dx.doi.org/10.1152/ajpcell.1992.262.2.c396.

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Rabbit polyclonal antibodies have been raised against high-performance liquid chromatography purified synthetic peptides corresponding to two discrete regions of the cystic fibrosis transmembrane regulator (CFTR) protein: the R-domain (residues 785-796) and the extreme COOH-terminus (residues 1467-1480). Antibodies (Ab) to each of these peptides were affinity purified either by passage over a peptide-derivatized agarose matrix (Ab 785) to produce monospecific polyclonal antibodies or by protein A affinity chromatography to purify the immunoglobulin G1 fraction free of other serum proteins (Ab 1467). These antibodies recognize a candidate CFTR protein in the colonic cell line T84, as determined by Western blot analysis and by immunoprecipitation and labeling of the precipitate with [gamma-32P]ATP in the presence of protein kinase A. Both antibodies precipitated CFTR-related polypeptides from four mammalian cell types (HeLa, Bsc-40, HEp-2, and Chinese hamster ovary cells) transfected with the full-length CFTR cDNA clone using a vaccinia T7 protein expression system. Similar results were observed using a yeast CFTR expression system. In each case the Mr values of the bands observed were consistent with that expected for the CFTR protein. These antibodies should be useful probes for the immunocytochemical localization, immunoaffinity purification, and ultimately the functional characterization of the CFTR protein.

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24

Rosenberg, Mark F., Liam P. O'Ryan, Guy Hughes, Zhefeng Zhao, Luba A. Aleksandrov, John R. Riordan, and Robert C. Ford. "The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)." Journal of Biological Chemistry 286, no. 49 (September 19, 2011): 42647–54. http://dx.doi.org/10.1074/jbc.m111.292268.

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25

V. Carey, Hannah. "Immune regulation of cystic fibrosis transmembrane regulator." Gastroenterology 109, no. 2 (August 1995): 630–31. http://dx.doi.org/10.1016/0016-5085(95)90359-3.

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26

Pranke, Iwona M., and Isabelle Sermet-Gaudelus. "Biosynthesis of cystic fibrosis transmembrane conductance regulator." International Journal of Biochemistry & Cell Biology 52 (July 2014): 26–38. http://dx.doi.org/10.1016/j.biocel.2014.03.020.

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27

Virant-Young, Deborah, Justin Thomas, Sarah Woiderski, Michelle Powers, Joseph Carlier, James McCarty, Tyler Kupchick, and Anthony Larder. "Cystic Fibrosis: A Novel Pharmacologic Approach to Cystic Fibrosis Transmembrane Regulator Modulation Therapy." Journal of the American Osteopathic Association 115, no. 9 (September 1, 2015): 546. http://dx.doi.org/10.7556/jaoa.2015.112.

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28

Becq, Frédéric. "Cystic Fibrosis Transmembrane Conductance Regulator Modulators for Personalized Drug Treatment of Cystic Fibrosis." Drugs 70, no. 3 (February 2010): 241–59. http://dx.doi.org/10.2165/11316160-000000000-00000.

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29

Bombieri, Cristina, Manuela Seia, and Carlo Castellani. "Genotypes and Phenotypes in Cystic Fibrosis and Cystic Fibrosis Transmembrane Regulator–Related Disorders." Seminars in Respiratory and Critical Care Medicine 36, no. 02 (March 31, 2015): 180–93. http://dx.doi.org/10.1055/s-0035-1547318.

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30

Pettit, Rebecca S. "Cystic Fibrosis Transmembrane Conductance Regulator–Modifying Medications: The Future of Cystic Fibrosis Treatment." Annals of Pharmacotherapy 46, no. 7-8 (July 2012): 1065–75. http://dx.doi.org/10.1345/aph.1r076.

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31

McIntosh, Iain, and Garry R. Cutting. "Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis." FASEB Journal 6, no. 10 (July 1992): 2775–82. http://dx.doi.org/10.1096/fasebj.6.10.1378801.

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32

Coutinho, Cyntia Arivabeni de Araujo Correia, Fernando Augusto de Lima Marson, Antonio Fernando Ribeiro, Jose Dirceu Ribeiro, and Carmen Silvia Bertuzzo. "Cystic fibrosis transmembrane conductance regulator mutations at a referral center for cystic fibrosis." Jornal Brasileiro de Pneumologia 39, no. 5 (September 2013): 555–61. http://dx.doi.org/10.1590/s1806-37132013000500005.

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OBJECTIVE: To determine the frequency of six mutations (F508del, G542X, G551D, R553X, R1162X, and N1303K) in patients with cystic fibrosis (CF) diagnosed, at a referral center, on the basis of abnormal results in two determinations of sweat sodium and chloride concentrations. METHODS: This was a cross-sectional study involving 70 patients with CF. The mean age of the patients was 12.38 ± 9.00 years, 51.43% were female, and 94.29% were White. Mutation screening was performed with polymerase chain reaction (for F508del), followed by enzymatic digestion (for other mutations). Clinical analysis was performed on the basis of gender, age, ethnicity, pulmonary/gastrointestinal symptoms, and Shwachman-Kulczycki (SK) score. RESULTS: All of the patients showed pulmonary symptoms, and 8 had no gastrointestinal symptoms. On the basis of the SK scores, CF was determined to be mild, moderate, and severe in 22 (42.3%), 17 (32.7%), and 13 (25.0%) of the patients, respectively. There was no association between F508del mutation and disease severity by SK score. Of the 140 alleles analyzed, F508del mutation was identified in 70 (50%). Other mutations (G542X, G551D, R553X, R1162X, and N1303K) were identified in 12 (7.93%) of the alleles studied. In F508del homozygous patients with severe disease, the OR was 0.124 (95% CI: 0.005-0.826). CONCLUSIONS: In 50% of the alleles studied, the molecular diagnosis of CF was confirmed by identifying a single mutation (F508del). If we consider the analysis of the six most common mutations in the Brazilian population (including F508del), the molecular diagnosis was confirmed in 58.57% of the alleles studied.

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33

Derichs, N. "Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis." European Respiratory Review 22, no. 127 (February 28, 2013): 58–65. http://dx.doi.org/10.1183/09059180.00008412.

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34

Sergeev, Valentine, Frank Y. Chou, Grace Y. Lam, Christopher Michael Hamilton, Pearce G. Wilcox, and Bradley S. Quon. "The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis." Annals of the American Thoracic Society 17, no. 2 (February 2020): 147–54. http://dx.doi.org/10.1513/annalsats.201909-671cme.

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35

Chiriac, Anca, Laura Trandafir, Cristian Podoleanu, and Simona Stolnicu. "Cutaneous Manifestations of Cystic Fibrosis." Journal of Interdisciplinary Medicine 3, no. 1 (March 1, 2018): 39–44. http://dx.doi.org/10.2478/jim-2018-0005.

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Abstract Cystic fibrosis (CF) is an autosomal recessive affliction triggered by genetic mutations in the cystic fibrosis transmembrane conductance regulator. The lung and pancreas are the most frequently affected organs in cystic fibrosis, cutaneous involvement is undervalued and underdiag-nosed. Skin lesions observed in patients diagnosed with cystic fibrosis are not well known and can create confusions with other dermatological diseases. The diagnosis of cutaneous lesions as signs of cystic fibrosis by pediatricians or dermatologists, despite their overlapping with different nutritional deficiencies, would allow earlier diagnosis and proper treatment and could improve quality of life and outcomes.

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36

Tector, Matthew, and F. Ulrich Hartl. "An unstable transmembrane segment in the cystic fibrosis transmembrane conductance regulator." EMBO Journal 18, no. 22 (November 15, 1999): 6290–98. http://dx.doi.org/10.1093/emboj/18.22.6290.

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37

Ren, Clement L., Rebecca L. Morgan, Christopher Oermann, Helaine E. Resnick, Cynthia Brady, Annette Campbell, Richard DeNagel, et al. "Cystic Fibrosis Foundation Pulmonary Guidelines. Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients with Cystic Fibrosis." Annals of the American Thoracic Society 15, no. 3 (March 2018): 271–80. http://dx.doi.org/10.1513/annalsats.201707-539ot.

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38

Coltrera, Marc D., Susan M. Mathison, Tracy A. Goodpaster, and Allen M. Gown. "Abnormal Expression of the Cystic Fibrosis Transmembrane Regulator in Chronic Sinusitis in Cystic Fibrosis and Non-Cystic Fibrosis Patients." Annals of Otology, Rhinology & Laryngology 108, no. 6 (June 1999): 576–81. http://dx.doi.org/10.1177/000348949910800609.

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39

Perry, L. A., J. C. Penny-Dimri, A. A. Aslam, T. W. Lee, and K. W. Southern. "Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease." Paediatric Respiratory Reviews 22 (March 2017): 47–49. http://dx.doi.org/10.1016/j.prrv.2016.10.005.

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40

Sloane, Peter A., and Steven M. Rowe. "Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis." Current Opinion in Pulmonary Medicine 16, no. 6 (November 2010): 591–97. http://dx.doi.org/10.1097/mcp.0b013e32833f1d00.

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41

Cuyx, Senne, and Kris De Boeck. "Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis." Seminars in Respiratory and Critical Care Medicine 40, no. 06 (October 28, 2019): 762–74. http://dx.doi.org/10.1055/s-0039-1696664.

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AbstractDetailed knowledge of how mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene disturb the trafficking or function of the CFTR protein and the use of high-throughput drug screens have allowed novel therapeutic strategies for cystic fibrosis (CF). The main goal of treatment is slowly but surely shifting from symptomatic management to targeting the underlying CFTR defect to halt disease progression and even to prevent occurrence of CF complications. CFTR potentiators for patients with class III mutations, mutation R117H (and in United States also for patients with specific residual function mutations) and the combination of a CFTR modulator plus a potentiator for patients homozygous for F508del, are the two classes of modulators that are in use in the clinic. Approval of these therapeutics has progressively expanded to include both younger patients and a wider range of CFTR mutations. For a significant proportion of patients with CF, current treatment is however still insufficient or unavailable.This review provides an overview of the clinical trial results and the real-life efficacy data of approved CFTR modulators. In addition, we discuss the entire pipeline of CFTR modulators: novel potentiators and correctors, amplifiers, stabilizers, and read-through agents. Furthermore, we discuss other strategies to improve CFTR function like nonsense-mediated decay inhibitors, modified transfer ribonucleic acids, antisense oligonucleotides, and genetic therapies.CFTR modulators are already changing the face of CF and the pipeline of new therapies continues to be exciting.

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42

Yang, Hong, and Tonghui Ma. "F508del-cystic fibrosis transmembrane regulator correctors for treatment of cystic fibrosis: a patent review." Expert Opinion on Therapeutic Patents 25, no. 9 (May 14, 2015): 991–1002. http://dx.doi.org/10.1517/13543776.2015.1045878.

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43

Fredj, Sondess Hadj, Taïeb Messaoud, Carine Templin, Marie des Georges, Slaheddine Fattoum, and Mireille Claustres. "Cystic Fibrosis Transmembrane Conductance Regulator Mutation Spectrum in Patients with Cystic Fibrosis in Tunisia." Genetic Testing and Molecular Biomarkers 13, no. 5 (October 2009): 577–81. http://dx.doi.org/10.1089/gtmb.2009.0028.

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44

Fukuda, Ryosuke, and Tsukasa Okiyoneda. "Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitylation as a Novel Pharmaceutical Target for Cystic Fibrosis." Pharmaceuticals 13, no. 4 (April 22, 2020): 75. http://dx.doi.org/10.3390/ph13040075.

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Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene decrease the structural stability and function of the CFTR protein, resulting in cystic fibrosis. Recently, the effect of CFTR-targeting combination therapy has dramatically increased, and it is expected that add-on drugs that modulate the CFTR surrounding environment will further enhance their effectiveness. Various interacting proteins have been implicated in the structural stability of CFTR and, among them, molecules involved in CFTR ubiquitylation are promising therapeutic targets as regulators of CFTR degradation. This review focuses on the ubiquitylation mechanism that contributes to the stability of mutant CFTR at the endoplasmic reticulum (ER) and post-ER compartments and discusses the possibility as a pharmacological target for cystic fibrosis (CF).

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45

Marshall, W. S., and T. D. Singer. "Cystic fibrosis transmembrane conductance regulator in teleost fish." Biochimica et Biophysica Acta (BBA) - Biomembranes 1566, no. 1-2 (November 2002): 16–27. http://dx.doi.org/10.1016/s0005-2736(02)00584-9.

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46

Sosnay, Patrick R., Karen S. Raraigh, and Ronald L. Gibson. "Molecular Genetics of Cystic Fibrosis Transmembrane Conductance Regulator." Pediatric Clinics of North America 63, no. 4 (August 2016): 585–98. http://dx.doi.org/10.1016/j.pcl.2016.04.002.

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47

Picciotto, M. R., J. A. Cohn, G. Bertuzzi, P. Greengard, and A. C. Nairn. "Phosphorylation of the cystic fibrosis transmembrane conductance regulator." Journal of Biological Chemistry 267, no. 18 (June 1992): 12742–52. http://dx.doi.org/10.1016/s0021-9258(18)42339-3.

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48

Villamizar, Olga, Shafagh A. Waters, Tristan Scott, Sheena Saayman, Nicole Grepo, Ryan Urak, Alicia Davis, Adam Jaffe, and Kevin V. Morris. "Targeted Activation of Cystic Fibrosis Transmembrane Conductance Regulator." Molecular Therapy 27, no. 10 (October 2019): 1737–48. http://dx.doi.org/10.1016/j.ymthe.2019.07.002.

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49

Eskandarani, H. A. "Cystic Fibrosis Transmembrane Regulator Gene Mutations in Bahrain." Journal of Tropical Pediatrics 48, no. 6 (December 1, 2002): 348–50. http://dx.doi.org/10.1093/tropej/48.6.348.

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50

Abraham, E. H. "Cystic Fibrosis Transmembrane Conductance Regulator and Adenosine Triphosphate." Science 275, no. 5304 (February 28, 1997): 1324–26. http://dx.doi.org/10.1126/science.275.5304.1324.

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