Page last updated: 2024-10-18

glycine and Cystic Fibrosis of Pancreas

glycine has been researched along with Cystic Fibrosis of Pancreas in 34 studies

Research Excerpts

ExcerptRelevanceReference
"The effect of taurine supplementation (30 to 40 mg/kg/24 hr) on fat absorption and related measurements was examined in 21 preadolescent children with cystic fibrosis (CF) using a 12-month double-blind crossover technique."9.06Taurine supplementation, fat absorption, and growth in cystic fibrosis. ( Davidson, GP; Robb, TA; Thompson, GN, 1987)
"Bile acid taurine deficiency is common in cystic fibrosis (CF) and is thought to be associated with impaired fat absorption."7.67Assessment of taurine deficiency in cystic fibrosis. ( Thompson, GN, 1988)
"The effect of taurine supplementation (30 to 40 mg/kg/24 hr) on fat absorption and related measurements was examined in 21 preadolescent children with cystic fibrosis (CF) using a 12-month double-blind crossover technique."5.06Taurine supplementation, fat absorption, and growth in cystic fibrosis. ( Davidson, GP; Robb, TA; Thompson, GN, 1987)
"VX-770 (Ivacaftor) has been approved for clinical usage in cystic fibrosis patients with several CFTR mutations."3.83Potentiators exert distinct effects on human, murine, and Xenopus CFTR. ( Cui, G; Imhoff, BR; Infield, DT; Khazanov, N; McCarty, NA; Senderowitz, H; Stauffer, BB, 2016)
" The mutation, a cysteine for glycine substitution at residue 480 (G480C), was detected in a pancreatic insufficient, African-American, cystic fibrosis (CF) patient."3.69Missense mutation (G480C) in the CFTR gene associated with protein mislocalization but normal chloride channel activity. ( Cohn, JA; Cole, JL; Cutting, GR; Dawson, DC; Macek, M; Mansoura, MK; Smit, LS; Strong, TV; Wilkinson, DJ; Wood, DL, 1995)
"The glycine-to-aspartic acid missense mutation at codon 551 (G551D), which is within the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator (CFTR), is the third most common cystic fibrosis (CF) mutation, with a worldwide frequency of 3."3.68Cystic fibrosis patients bearing both the common missense mutation Gly----Asp at codon 551 and the delta F508 mutation are clinically indistinguishable from delta F508 homozygotes, except for decreased risk of meconium ileus. ( Brock, DJ; Corey, M; Durie, P; Hamosh, A; Keston, M; King, TM; Levison, H; McIntosh, I; Rosenstein, BJ; Tsui, LC, 1992)
"To evaluate the relative efficacy of nonelemental versus semielemental enteral supplements for nutritional rehabilitation of cystic fibrosis (CF) patients, whole-body protein turnover using the [15N]glycine method was studied in nine malnourished CF patients during enteral feedings, in a block design study comparing a semielemental formula (Criticare), a higher protein density but nonelemental formula (Traumacal) (T), and a nonelemental formula that had been modified to become isocaloric and isonitrogenous to the semielemental formula (modified Traumacal, MT)."3.68Protein turnover in malnourished patients with cystic fibrosis: effects of elemental and nonelemental nutritional supplements. ( Cleghorn, GJ; Holt, TL; Pelekanos, JT; Shepherd, RW; Ward, LC, 1990)
"Bile acid taurine deficiency is common in cystic fibrosis (CF) and is thought to be associated with impaired fat absorption."3.67Assessment of taurine deficiency in cystic fibrosis. ( Thompson, GN, 1988)
"CF (cystic fibrosis) is caused by mutations in CFTR (CF transmembrane conductance regulator), which cause its mistrafficking and/or dysfunction as a regulated chloride channel on the apical surface of epithelia."1.35Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease. ( Bear, CE; Li, C; Pasyk, S; Ramjeesingh, M, 2009)
"Bile is lithogenic in untreated cystic fibrosis and responds to pancreatic enzymes."1.26Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes. ( Combes, JC; Lasalle, R; Mégevand, A; Morin, CL; Nusslé, D; Roy, CC; Weber, AM, 1977)

Research

Studies (34)

TimeframeStudies, this research(%)All Research%
pre-19907 (20.59)18.7374
1990's8 (23.53)18.2507
2000's8 (23.53)29.6817
2010's9 (26.47)24.3611
2020's2 (5.88)2.80

Authors

AuthorsStudies
Tabori, H1
Schneider, J1
Lüth, S1
Zagoya, C1
Barucha, A1
Lehmann, T1
Kauf, E1
Barth, A1
Mainz, JG1
Zagefka, H1
Huynh, HP1
Senger, AR1
Derington, CG1
Colantonio, LD1
Herrick, JS1
Cook, J1
King, JB1
Rosenson, RS1
Poudel, B1
Monda, KL1
Navar, AM1
Mues, KE1
Stevens, VW1
Nelson, RE1
Vanneman, ME1
Muntner, P1
Bress, AP1
Ma, B1
Ren, G1
Xu, J1
Yin, C1
Shi, Y1
Rahsepar, AA1
Bluemke, DA1
Habibi, M1
Liu, K1
Kawel-Boehm, N1
Ambale-Venkatesh, B1
Fernandes, VRS1
Rosen, BD1
Lima, JAC1
Carr, JC1
Freitag, TM1
Chen-Sankey, JC1
Duarte, DA1
Ramsey, MW1
Choi, K1
Winkler-Heil, R1
Hussain, M1
Hofmann, W1
Nicotera, AG1
Dicanio, D1
Pironti, E1
Bonsignore, M1
Cafeo, A1
Efthymiou, S1
Mondello, P1
Salpietro, V1
Houlden, H1
Di Rosa, G1
Hayes-Ryan, D1
O'Donoghue, K1
McCarthy, C1
Totorika, A1
Meaney, S1
Pang, RD1
Dormanesh, A1
Hoang, Y1
Chu, M1
Allem, JP1
Girón-Ortega, JA1
Márquez-Coello, M1
Gutiérrez-Saborido, D1
Arizcorreta, A1
Cuesta-Sancho, S1
Girón-González, JA1
Dovrat, G1
Pevzner, S1
Berthon, C1
Lerner, A1
Maimon, E1
Vainer, R1
Karpasas, M1
Ben-Elyiahu, Y1
Moisy, P1
Bettelheim, A1
Zilbermann, I1
Vanden Broeck, SMP1
Nelson, DJ1
Collado, A1
Falivene, L1
Cavallo, L1
Cordes, DB1
Slawin, AMZ1
Van Hecke, K1
Nahra, F1
Cazin, CSJ1
Nolan, SP1
Kranidiotis-Hisatomi, N1
Yi, H1
Oestreich, M1
Kwiezinski, C1
Weller, C1
van Pinxteren, D1
Brüggemann, M1
Mertes, S1
Stratmann, F1
Herrmann, H1
Treggiari, D1
Tridello, G1
Menin, L1
Borruso, A1
Pintani, E1
Iansa, P1
Cipolli, M1
Melotti, P1
Ren, L1
Liu, L3
Shu, X1
Lin, W1
Yang, P1
Chen, J1
Teo, KL1
Xiao, F1
Wang, H1
Yao, T1
Zhao, X1
Yang, X1
Yu, DYW1
Rogach, AL1
Bordet, A1
Leitner, W1
Gao, S1
Xia, F1
Li, B2
Abdul Razak, IB1
Liu, Y1
Lu, K1
Brown, DE1
Wang, R1
Cheng, Y1
Ni, S1
Qu, H1
Xing, H1
Xu, Z1
Zhu, X1
Yuan, M1
Wang, L1
Yu, J1
Li, Y1
Yang, L1
Liu, H1
Cao, L1
Zhang, S1
Zhao, D1
Yan, T1
Yang, G1
Lin, Z1
Luo, M1
Ye, N1
Lee, SW1
Carnicelli, J1
Getya, D1
Gitsov, I1
Phillips, KS1
Ren, D1
Grützmacher, PG1
Suarez, S1
Tolosa, A1
Gachot, C1
Song, G1
Wang, B1
Presser, V1
Mücklich, F1
Anasori, B1
Rosenkranz, A1
Demireva, M1
Armentrout, PB1
Feng, D1
Cao, K1
He, ZZ1
Knibbs, LD1
Jalaludin, B1
Leskinen, A1
Roponen, M1
Komppula, M1
Jalava, P1
Guo, PY1
Xu, SL1
Yang, BY1
Hu, L1
Zeng, XW1
Chen, G1
Yu, HY1
Lin, L1
Dong, G1
Machulkin, AE1
Shafikov, RR1
Uspenskaya, AA1
Petrov, SA1
Ber, AP1
Skvortsov, DA1
Nimenko, EA1
Zyk, NU1
Smirnova, GB1
Pokrovsky, VS1
Abakumov, MA1
Saltykova, IV1
Akhmirov, RT1
Garanina, AS1
Polshakov, VI1
Saveliev, OY1
Ivanenkov, YA1
Aladinskaya, AV1
Finko, AV1
Yamansarov, EU1
Krasnovskaya, OO1
Erofeev, AS1
Gorelkin, PV1
Dontsova, OA1
Beloglazkina, EK1
Zyk, NV1
Khazanova, ES1
Majouga, AG1
Zheng, YK1
Su, BJ1
Wang, YQ1
Wang, HS1
Liao, HB1
Liang, D1
Shataer, D1
Li, J1
Duan, XM1
Xin, XL1
Aisa, HA1
Allu, SR1
Ravotto, L1
Troxler, T1
Vinogradov, SA1
Cheruku, RR1
Tracy, EC1
Tabaczynski, W1
Missert, JR1
Baumann, H1
Pandey, RK1
Rohde, JM1
Karavadhi, S1
Pragani, R1
Fang, Y1
Zhang, W1
McIver, A1
Zheng, H1
Liu, Q1
Davis, MI1
Urban, DJ1
Lee, TD1
Cheff, DM1
Hollingshead, M1
Henderson, MJ1
Martinez, NJ1
Brimacombe, KR1
Yasgar, A1
Zhao, W1
Klumpp-Thomas, C1
Michael, S1
Covey, J1
Moore, WJ1
Stott, GM1
Li, Z1
Simeonov, A1
Jadhav, A1
Frye, S1
Hall, MD1
Shen, M1
Wang, X1
Patnaik, S1
Boxer, MB1
Zhang, H2
Cheng, C1
Huang, B1
Chen, R1
Huang, Y1
Chen, H1
Pei, W1
Katahara, S1
Sugiyama, Y1
Yamane, M1
Komiya, Y1
Sato, T1
Chida, N1
Vargas, MH1
Del-Razo-Rodríguez, R1
López-García, A1
Lezana-Fernández, JL1
Chávez, J1
Furuya, MEY1
Marín-Santana, JC1
Adam, D1
Bilodeau, C1
Sognigbé, L1
Maillé, É1
Ruffin, M1
Brochiero, E1
Edlund, A1
Esguerra, JL1
Wendt, A1
Flodström-Tullberg, M1
Eliasson, L1
Cui, G1
Khazanov, N1
Stauffer, BB1
Infield, DT1
Imhoff, BR1
Senderowitz, H1
McCarty, NA1
Pasyk, S1
Li, C1
Ramjeesingh, M1
Bear, CE1
Hagins, JM1
Locy, R1
Silo-Suh, L1
Masvidal, L1
Giménez, J1
Ramos, MD1
Domingo, C1
Farré, A1
Bassas, L1
Casals, T1
Mills, AD1
Yoo, C1
Butler, JD1
Yang, B1
Verkman, AS3
Kurth, MJ1
Dubarry, N1
Du, W1
Lane, D1
Pasta, F1
Patrick, AE1
Karamyshev, AL1
Millen, L1
Thomas, PJ1
Gontijo-Amaral, C1
Guimarães, EV1
Camargos, P1
Synder, D1
Tradtrantip, L1
Thiagarajah, JR1
Anderson, MO1
KAESER, H1
COTTIER, P1
ANTENER, I1
Melin, P1
Thoreau, V1
Norez, C1
Bilan, F1
Kitzis, A1
Becq, F1
Decaestecker, K1
Decaestecker, E1
Castellani, C1
Jaspers, M1
Cuppens, H1
De Boeck, K1
Lukacs, GL1
Galietta, LJ1
Smit, LS1
Strong, TV1
Wilkinson, DJ1
Macek, M1
Mansoura, MK1
Wood, DL1
Cole, JL1
Cutting, GR1
Cohn, JA1
Dawson, DC1
Morrison, RT1
Steuart, RD1
Chaun, H1
Fortunati, E1
Bout, A1
Zanta, MA1
Valerio, D1
Scarpa, M2
Cartault, F1
Steffann, J1
Vidaud, D1
Bousquet, S1
Lesure, F1
Renouil, M1
McDonell, N1
Feingold, J1
Beldjord, C1
Bienvenu, T1
Picci, L1
Cameran, M1
Olante, P1
Zacchello, F1
Thomas, GR1
Costelloe, EA1
Lunn, DP1
Stacey, KJ1
Delaney, SJ1
Passey, R1
McGlinn, EC1
McMorran, BJ1
Ahadizadeh, A1
Geczy, CL1
Wainwright, BJ1
Hume, DA1
Foster, JA1
Ramsden, WH1
Conway, SP1
Taylor, JM1
Etherington, C1
Lefebvre, D1
Ratelle, S1
Chartrand, L1
Roy, CC2
Weber, AM1
Morin, CL1
Combes, JC1
Nusslé, D1
Mégevand, A1
Lasalle, R1
Hamosh, A1
King, TM1
Rosenstein, BJ1
Corey, M1
Levison, H1
Durie, P1
Tsui, LC1
McIntosh, I1
Keston, M1
Brock, DJ1
Franciolini, F1
Petris, A1
Pelekanos, JT1
Holt, TL1
Ward, LC1
Cleghorn, GJ1
Shepherd, RW1
Thompson, GN3
Robb, TA1
Davidson, GP1
Lane, JI1
Sacks, D1
Winn, RS1
Kershner, MS1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Controlled and Randomized Clinical Trial for Evaluating the Effect of a Supplement of Glycine as Adjuvant in the Treatment of COVID-19 Pneumonia in Patients Initiating Mechanical Ventilation[NCT04443673]59 participants (Actual)Interventional2020-06-15Terminated (stopped due to An interim analysis showed no difference in major outcomes (n=35 glycine and n=24 control participants))
Evaluation of the Capability of a Glycine Oral Supplement for Diminishing Bronchial Inflammation in Children With Cystic Fibrosis[NCT01417481]Phase 213 participants (Actual)Interventional2012-03-31Terminated (stopped due to Some of the researchers finished their participation in the study.)
Characterization of β-cell Function and Insulin Sensitivity in Pre-transplant Patients With Cystic Fibrosis[NCT04379726]150 participants (Anticipated)Observational2020-07-01Not yet recruiting
Phase II Study: LYM-X-SORB™, an Organized Lipid Matrix: Fatty Acids and Choline in CF[NCT00406536]Phase 2110 participants (Actual)Interventional2007-01-31Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Changes in Serum Concentration of Inflammatory Biomarkers (TNF-alpha)

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). Then, percentages were log-transformed to adjust to a normal distribution. (NCT01417481)
Timeframe: 8 weeks

Interventionlog (percent change) (Mean)
Glycine-0.3908
Placebo0.2035

Changes in Sputum Concentration of Inflammatory Biomarkers (G-CSF)

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). Then, percentage change was log-transformed to adjust to a normal distribution. (NCT01417481)
Timeframe: 8 weeks

Interventionlog (percent change) (Mean)
Glycine-0.0819
Placebo0.1668

Changes in Sputum Concentration of Inflammatory Biomarkers (IL-6)

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). Then, percentage change was log-transformed to adjust to a normal distribution. (NCT01417481)
Timeframe: 8 weeks

Interventionlog (percent change) (Mean)
Glycine-0.00007
Placebo0.1739

Changes in Clinical Data Scores (Other Than Sputum Production, Dyspnea and Global Symptoms)

"To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]).~Each respiratory symptom (Cough severity, Sputum features, Appetite, Dyspnea, and Energy perception) was evaluated in a 5-options Likert scale, ranging from 1 (better) to 5 (worse). The total score was computed by the simple sum of the five symptoms." (NCT01417481)
Timeframe: 8 weeks

,
InterventionPercentage of baseline (Mean)
Cough questionnaire scoreAppetite questionnaire scoreEnergy questionnaire scoreBody weightHeightHeart rateRespiratory rateTemperature
Glycine81.189.184.6101.6100.5103.594.8100.0
Placebo89.1132.1111.5103.6100.598.1109.0100.1

Changes in FEV1, FEF25, and FEFmax

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). (NCT01417481)
Timeframe: 8 weeks

,
InterventionPercentage of baseline (Mean)
Forced expiratory volume at first second (FEV1)Forced expiratory flow at 25%FVC (FEF25)Maximal forced expiratory flow (FEFmax, PEFR)
Glycine109.7133.9115.3
Placebo91.483.391.2

Changes in Other Spirometric Variables

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). (NCT01417481)
Timeframe: 8 weeks

,
InterventionPercentage of baseline (Mean)
Forced vital capacity (FVC)Forced expiratory flow at 75%FVC (FEF75)
Glycine104.1111.8
Placebo100.6108.9

Changes in Pulse Oximetry, FEV1/FVC, and FEF50.

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). (NCT01417481)
Timeframe: 8 weeks

,
InterventionPercentage of baseline (Mean)
Peripheral oxygen saturation (SpO2)FEV1/FVCForced expiratory flow at 50%FVC (FEF50)
Glycine105.2105.2115.5
Placebo98.994.993.1

Changes in Score for Sputum Production, Dyspnea and Global Symptoms

"To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]).~In the symptoms questionnaire, each respiratory symptom (Cough severity, Sputum features, Appetite, Dyspnea, and Energy perception) was evaluated in a 5-options Likert scale, ranging from 1 (better) to 5 (worse). The total score was computed by the simple sum of the five symptoms." (NCT01417481)
Timeframe: 8 weeks

,
InterventionPercentage of baseline (Mean)
Sputum questionnaire scoreDyspnea questionnaire scoreTotal questionnaire score
Glycine82.075.677.7
Placebo102.6103.898.7

Changes in Serum Concentration of Inflammatory Biomarkers (Other Than TNF-alpha)

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). Then, percentages were log-transformed to adjust to a normal distribution. (NCT01417481)
Timeframe: 8 weeks

,
Interventionlog (percent change) (Mean)
MyeloperoxidaseIL-1IL-4IL-6IL-7IL-8IL-12IL-13G-CSFIFN-gammaMCP-1MIP-1beta
Glycine-0.4361-0.16350.29640.00850.0356-0.14660.3203-0.0561-0.07760.3272-0.08360.0330
Placebo-0.2906-0.03520.14700.22550.0819-0.23640.26030.19530.22720.36390.0472-0.0608

Changes in Sputum Concentration of Inflammatory Biomarkers (Other Than IL-6 and G-CSF)

To correct for the baseline variability, all measurements were expressed as percentage of baseline (value at week 8 with respect to baseline value [beginning of the glycine or placebo period, respectively]). Then, percentage change was log-transformed to adjust to a normal distribution. (NCT01417481)
Timeframe: 8 weeks

,
Interventionlog (percent change) (Mean)
MyeloperoxidaseIL-1IL-2IL-4IL-5IL-7IL-8IL-10IL-12IL-13IL-17IFN-gammaMCP-1MIP-1betaTNF-alphaGM-CSF
Glycine0.1294-0.09180.0233-0.01610.24980.0611-0.08240.05490.16750.16300.06800.02480.0042-0.03030.0412-0.0538
Placebo0.0669-0.0102-0.02740.05220.13040.13870.05420.00740.06770.09530.11400.06490.26080.09770.1568-0.0822

Reviews

3 reviews available for glycine and Cystic Fibrosis of Pancreas

ArticleYear
CFTR inhibitors.
    Current pharmaceutical design, 2013, Volume: 19, Issue:19

    Topics: Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Discovery; Glyci

2013
CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis.
    Current pharmaceutical design, 2006, Volume: 12, Issue:18

    Topics: Animals; Antidiarrheals; Biosensing Techniques; Cell Line; Chlorides; Cystic Fibrosis; Cystic Fibros

2006
Chloride channels of biological membranes.
    Biochimica et biophysica acta, 1990, May-07, Volume: 1031, Issue:2

    Topics: Animals; Biological Transport; Calcium; Cell Membrane; Chloride Channels; Chlorides; Cystic Fibrosis

1990

Trials

3 trials available for glycine and Cystic Fibrosis of Pancreas

ArticleYear
Effect of oral glycine on the clinical, spirometric and inflammatory status in subjects with cystic fibrosis: a pilot randomized trial.
    BMC pulmonary medicine, 2017, Dec-15, Volume: 17, Issue:1

    Topics: Administration, Oral; Adolescent; Anti-Inflammatory Agents; Biomarkers; Child; Cross-Over Studies; C

2017
Effect of oral glycine on the clinical, spirometric and inflammatory status in subjects with cystic fibrosis: a pilot randomized trial.
    BMC pulmonary medicine, 2017, Dec-15, Volume: 17, Issue:1

    Topics: Administration, Oral; Adolescent; Anti-Inflammatory Agents; Biomarkers; Child; Cross-Over Studies; C

2017
Effect of oral glycine on the clinical, spirometric and inflammatory status in subjects with cystic fibrosis: a pilot randomized trial.
    BMC pulmonary medicine, 2017, Dec-15, Volume: 17, Issue:1

    Topics: Administration, Oral; Adolescent; Anti-Inflammatory Agents; Biomarkers; Child; Cross-Over Studies; C

2017
Effect of oral glycine on the clinical, spirometric and inflammatory status in subjects with cystic fibrosis: a pilot randomized trial.
    BMC pulmonary medicine, 2017, Dec-15, Volume: 17, Issue:1

    Topics: Administration, Oral; Adolescent; Anti-Inflammatory Agents; Biomarkers; Child; Cross-Over Studies; C

2017
Oral magnesium supplementation in children with cystic fibrosis improves clinical and functional variables: a double-blind, randomized, placebo-controlled crossover trial.
    The American journal of clinical nutrition, 2012, Volume: 96, Issue:1

    Topics: Adolescent; Adult; Child; Cross-Over Studies; Cystic Fibrosis; Dietary Supplements; Double-Blind Met

2012
Taurine supplementation, fat absorption, and growth in cystic fibrosis.
    The Journal of pediatrics, 1987, Volume: 111, Issue:4

    Topics: Body Height; Body Weight; Child; Child, Preschool; Clinical Trials as Topic; Cystic Fibrosis; Dietar

1987

Other Studies

28 other studies available for glycine and Cystic Fibrosis of Pancreas

ArticleYear
Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with
    International journal of molecular sciences, 2022, Oct-18, Volume: 23, Issue:20

    Topics: Bile Acids and Salts; Cholic Acid; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regula

2022
    Journal of applied social psychology, 2021, Volume: 51, Issue:5

    Topics: Adult; Aged; Aged, 80 and over; Air Pollutants; Air Pollution; Animals; Anti-Bacterial Agents; Anti-

2021
CFTR rescue with VX-809 and VX-770 favors the repair of primary airway epithelial cell cultures from patients with class II mutations in the presence of Pseudomonas aeruginosa exoproducts.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2018, Volume: 17, Issue:6

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cells, Cultured; Chloride Channel Agonists; Cystic Fibr

2018
CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells.
    BMC medicine, 2014, May-28, Volume: 12

    Topics: Animals; Anoctamin-1; Calcium; Calcium Channels; Chloride Channels; Colforsin; Cyclic AMP; Cystic Fi

2014
Potentiators exert distinct effects on human, murine, and Xenopus CFTR.
    American journal of physiology. Lung cellular and molecular physiology, 2016, 08-01, Volume: 311, Issue:2

    Topics: Amino Acid Sequence; Aminophenols; Animals; Cells, Cultured; Chloride Channel Agonists; Cystic Fibro

2016
Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease.
    The Biochemical journal, 2009, Feb-15, Volume: 418, Issue:1

    Topics: Adenosine Triphosphatases; Adenosine Triphosphate; Animals; Cell Line; Cresols; Cystic Fibrosis; Cys

2009
Isocitrate lyase supplies precursors for hydrogen cyanide production in a cystic fibrosis isolate of Pseudomonas aeruginosa.
    Journal of bacteriology, 2009, Volume: 191, Issue:20

    Topics: Adaptation, Physiological; Carrier State; Cystic Fibrosis; D-Amino-Acid Oxidase; Gene Expression Reg

2009
The p.Arg258Gly mutation in intracellular loop 2 of CFTR is associated with CFTR-related disorders.
    Genetic testing and molecular biomarkers, 2009, Volume: 13, Issue:6

    Topics: Adult; Aged; Amino Acid Sequence; Amino Acid Substitution; Arginine; Cohort Studies; Cystic Fibrosis

2009
Design and synthesis of a hybrid potentiator-corrector agonist of the cystic fibrosis mutant protein DeltaF508-CFTR.
    Bioorganic & medicinal chemistry letters, 2010, Jan-01, Volume: 20, Issue:1

    Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Design; Glycine; Humans;

2010
Improved electrotransformation and decreased antibiotic resistance of the cystic fibrosis pathogen Burkholderia cenocepacia strain J2315.
    Applied and environmental microbiology, 2010, Volume: 76, Issue:4

    Topics: Base Sequence; Burkholderia cepacia complex; Culture Media; Cystic Fibrosis; DNA Methylation; DNA Pr

2010
Alteration of CFTR transmembrane span integration by disease-causing mutations.
    Molecular biology of the cell, 2011, Volume: 22, Issue:23

    Topics: Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endoplasmic Ret

2011
[Gluco-glycinuria, a new familial syndrome].
    Helvetica paediatrica acta, 1961, Volume: 16

    Topics: Body Fluids; Cystic Fibrosis; Glycine; Glycosuria; Humans; Kidney Diseases; Medical Records; Syndrom

1961
The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein.
    Biochemical pharmacology, 2004, Jun-15, Volume: 67, Issue:12

    Topics: Amino Acid Motifs; Animals; Aspartic Acid; COS Cells; Cystic Fibrosis; Cystic Fibrosis Transmembrane

2004
Genotype/phenotype correlation of the G85E mutation in a large cohort of cystic fibrosis patients.
    The European respiratory journal, 2004, Volume: 23, Issue:5

    Topics: Adolescent; Adult; Animals; Child; Child, Preschool; Chlorocebus aethiops; Cohort Studies; COS Cells

2004
Missense mutation (G480C) in the CFTR gene associated with protein mislocalization but normal chloride channel activity.
    Human molecular genetics, 1995, Volume: 4, Issue:2

    Topics: Animals; Base Sequence; Cells, Cultured; Chloride Channels; Cyclic AMP; Cysteine; Cystic Fibrosis; F

1995
Multifocal uptake of Tc-99m mebrofenin in dilated liver ductules in a patient with cystic fibrosis.
    Clinical nuclear medicine, 1995, Volume: 20, Issue:1

    Topics: Adult; Aniline Compounds; Bile Ducts, Intrahepatic; Cholestasis, Intrahepatic; Cystic Fibrosis; Dila

1995
In vitro and in vivo gene transfer to pulmonary cells mediated by cationic liposomes.
    Biochimica et biophysica acta, 1996, Apr-10, Volume: 1306, Issue:1

    Topics: 3T3 Cells; Adenoviridae; Animals; beta-Galactosidase; Bronchi; Cations; Cell Line; Cells, Cultured;

1996
Detection of more than 91% cystic fibrosis mutations in a sample of the population from Reunion Island and identification of two novel mutations (A309G, S1255L) and one novel polymorphism (L49L)
    Clinical genetics, 1998, Volume: 54, Issue:5

    Topics: Alanine; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; France; Glycine; Huma

1998
Identification of a D579G homozygote cystic fibrosis patient with pancreatic sufficiency and minor lung involvement. Mutations in brief no. 221. Online.
    Human mutation, 1999, Volume: 13, Issue:2

    Topics: Adult; Amino Acid Substitution; Aspartic Acid; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; F

1999
G551D cystic fibrosis mice exhibit abnormal regulation of inflammation in lungs and macrophages.
    Journal of immunology (Baltimore, Md. : 1950), 2000, Apr-01, Volume: 164, Issue:7

    Topics: Amino Acid Substitution; Animals; Aspartic Acid; Biomarkers; Bone Marrow Cells; Cell Movement; Cysti

2000
The role of IDA scintigraphy in the follow-up of liver disease in patients with cystic fibrosis.
    Nuclear medicine communications, 2002, Volume: 23, Issue:7

    Topics: Adolescent; Adult; Aniline Compounds; Child; Cystic Fibrosis; Female; Follow-Up Studies; Glycine; Hu

2002
Reduced microbial transformation of bile acids in cystic fibrosis.
    Experientia, 1977, May-15, Volume: 33, Issue:5

    Topics: Adolescent; Bile Acids and Salts; Chenodeoxycholic Acid; Child; Child, Preschool; Cholic Acids; Cyst

1977
Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes.
    The New England journal of medicine, 1977, Dec-15, Volume: 297, Issue:24

    Topics: Bile; Bile Acids and Salts; Child; Cholelithiasis; Cholesterol; Cholic Acids; Cystic Fibrosis; Femal

1977
Cystic fibrosis patients bearing both the common missense mutation Gly----Asp at codon 551 and the delta F508 mutation are clinically indistinguishable from delta F508 homozygotes, except for decreased risk of meconium ileus.
    American journal of human genetics, 1992, Volume: 51, Issue:2

    Topics: Adolescent; Aspartic Acid; Child; Child, Preschool; Codon; Cystic Fibrosis; Cystic Fibrosis Transmem

1992
Protein turnover in malnourished patients with cystic fibrosis: effects of elemental and nonelemental nutritional supplements.
    Journal of pediatric gastroenterology and nutrition, 1990, Volume: 10, Issue:3

    Topics: Adolescent; Child; Child Nutrition Disorders; Child Nutritional Physiological Phenomena; Child, Pres

1990
Failure of taurine to improve fat absorption in cystic fibrosis.
    Journal of inherited metabolic disease, 1988, Volume: 11 Suppl 2

    Topics: Adolescent; Child; Cystic Fibrosis; Female; Glycine; Humans; Intestinal Absorption; Lipid Metabolism

1988
Assessment of taurine deficiency in cystic fibrosis.
    Clinica chimica acta; international journal of clinical chemistry, 1988, Feb-15, Volume: 171, Issue:2-3

    Topics: Adolescent; Bile; Child; Child, Preschool; Cystic Fibrosis; Female; Glycine; Humans; Leukocytes; Liv

1988
A false-positive hepatobiliary scan in a patient with cystic fibrosis.
    Clinical nuclear medicine, 1988, Volume: 13, Issue:5

    Topics: Adult; Aniline Compounds; Biliary Tract; Cholecystitis; Cystic Fibrosis; False Positive Reactions; F

1988