Page last updated: 2024-09-03

cystic fibrosis transmembrane conductance regulator (505-511) and Cystic Fibrosis

cystic fibrosis transmembrane conductance regulator (505-511) has been researched along with Cystic Fibrosis in 14 studies

*Cystic Fibrosis: An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. [MeSH]

Research

Studies (14)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's1 (7.14)18.2507
2000's9 (64.29)29.6817
2010's4 (28.57)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Bompadre, SG; Kopeikin, Z; Yang, HY; Yuksek, Z1
Mayor, S1
Ittenbach, RF; McBride, MG; Paridon, SM; Schall, JI; Stallings, VA; Zemel, BS1
Cooper-Knock, J; Garrard, K; Lo, C; Martindale, J; Shaw, P; Williams, T1
Banks-Schlegel, SP; Guggino, WB1
Aebischer, CC; Keller, BM; Kraemer, R; Schöni, MH1
Cabello, GM; Cabello, PH; Fernandes, O; Llerena, JC; Lopez-Camelo, JS1
Nau, JY1
Ratjen, F1
Ahrens, RC; Aitken, ML; Hilliard, KA; Kelley, TJ; Konstan, MW; Launspach, J; McCarty, NA; Milgram, LJ; Regelmann, WE; Standaert, TA; Teresi, M; Tuthill, C; Weatherly, MR1
Brooks, JG; Detzer, M; Kahn, R; Lever, T; Maddock, J; O'Connor, GT; Quinton, HB; Robichaud, P1
Bush, A; Crowley, S1
Borowitz, D; Cimino, M; Simon, D1
Chan, HC; Dedman, JR; Hazarika, P; Kaetzel, MA; Nelson, DJ1

Reviews

2 review(s) available for cystic fibrosis transmembrane conductance regulator (505-511) and Cystic Fibrosis

ArticleYear
Macromolecular interactions and ion transport in cystic fibrosis.
    American journal of respiratory and critical care medicine, 2004, Oct-01, Volume: 170, Issue:7

    Topics: Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Endoplasmic Reticulum; Forecasting; Humans; Ion Transport; Macromolecular Substances; Mutation; Needs Assessment; Peptide Fragments; Protein Folding; Protein Interaction Mapping; Research

2004
New pulmonary therapies for cystic fibrosis.
    Current opinion in pulmonary medicine, 2007, Volume: 13, Issue:6

    Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Therapy; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Lung Diseases; Peptide Fragments; Sodium Channel Blockers; Treatment Outcome

2007

Trials

1 trial(s) available for cystic fibrosis transmembrane conductance regulator (505-511) and Cystic Fibrosis

ArticleYear
A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis.
    Pediatric pulmonology, 2002, Volume: 33, Issue:2

    Topics: Adolescent; Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Dose-Response Relationship, Drug; Female; Humans; Male; Membrane Potentials; Mutation, Missense; Nasal Mucosa; Peptide Fragments; Purinergic P1 Receptor Antagonists; Regression Analysis; Research Design; Sweat; Treatment Outcome; Xanthines

2002

Other Studies

11 other study(ies) available for cystic fibrosis transmembrane conductance regulator (505-511) and Cystic Fibrosis

ArticleYear
Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del-CFTR channels.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2014, Volume: 13, Issue:5

    Topics: Adenosine Triphosphate; Aminophenols; Aminopyridines; Benzodioxoles; Carrier Proteins; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; In Vitro Techniques; Intracellular Signaling Peptides and Proteins; Patch-Clamp Techniques; Peptide Fragments; Quinolones

2014
Drug combination that corrects deficient protein in cystic fibrosis improves lung function.
    BMJ (Clinical research ed.), 2015, May-19, Volume: 350

    Topics: Aminophenols; Aminopyridines; Benzodioxoles; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Drug Therapy, Combination; Humans; Peptide Fragments; Quinolones; Randomized Controlled Trials as Topic; Respiratory Function Tests

2015
Clinical and genetic correlates of exercise performance in young children with cystic fibrosis.
    Perceptual and motor skills, 2010, Volume: 110, Issue:3 Pt 2

    Topics: Anthropometry; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Exercise Test; Female; Genotype; Heterozygote; Humans; Male; Malnutrition; Nutritional Status; Peptide Fragments; Respiratory Function Tests

2010
Concurrent amyotrophic lateral sclerosis and cystic fibrosis supports common pathways of pathogenesis.
    Amyotrophic lateral sclerosis & frontotemporal degeneration, 2013, Volume: 14, Issue:5-6

    Topics: Adult; Amyotrophic Lateral Sclerosis; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA-Binding Proteins; Female; Humans; Mutation; Peptide Fragments; RNA Splicing

2013
Growth in prepubertal children with cystic fibrosis, homozygous for the Delta F508 mutation.
    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2003, Volume: 2, Issue:2

    Topics: Adolescent; Analysis of Variance; Body Height; Body Mass Index; Body Weight; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Forced Expiratory Volume; Genotype; Growth Disorders; Homozygote; Hospitals, Pediatric; Humans; Linear Models; Longitudinal Studies; Male; Maximal Midexpiratory Flow Rate; Mutation; Peptide Fragments; Retrospective Studies; Switzerland; Vital Capacity

2003
Haplotype distribution of and linkage disequilibrium between four polymorphic markers near the CFTR locus in Brazilian cystic fibrosis patients.
    Human biology, 2005, Volume: 77, Issue:6

    Topics: Africa South of the Sahara; Alleles; Brazil; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetics, Population; Haplotypes; Humans; Linkage Disequilibrium; Mediterranean Islands; Peptide Fragments; Polymorphism, Genetic

2005
[Prenatal testing for cystic fibrosis].
    Revue medicale suisse, 2006, Sep-27, Volume: 2, Issue:80

    Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Mutation; Peptide Fragments; Pregnancy; Prenatal Diagnosis

2006
Case-mix adjustment for evaluation of mortality in cystic fibrosis.
    Pediatric pulmonology, 2002, Volume: 33, Issue:2

    Topics: Age Factors; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Ethnicity; Female; Genotype; Humans; Infant; Male; Multivariate Analysis; Peptide Fragments; Proportional Hazards Models; Racial Groups; Registries; Risk Adjustment; Sex Factors; Socioeconomic Factors; Survival Analysis; United States

2002
Cystic fibrosis: keeping it in the family.
    Pediatric pulmonology, 2002, Volume: 33, Issue:2

    Topics: Adult; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Fertility; Homozygote; Humans; Infant; Male; Peptide Fragments; Phenotype; Pregnancy; Sweat

2002
Normal volume of distribution of tobramycin in a mother and daughter with a CFTR splice mutation (1717 - 1G --> A).
    Pediatric pulmonology, 2002, Volume: 33, Issue:4

    Topics: Adult; Anti-Bacterial Agents; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Mutation; Peptide Fragments; Tobramycin

2002
Antibody against a cystic fibrosis transmembrane conductance regulator-derived synthetic peptide inhibits anion currents in human colonic cell line T84.
    The Journal of biological chemistry, 1992, Apr-25, Volume: 267, Issue:12

    Topics: Anions; Antibodies; Biological Transport; Blotting, Western; Calcium; Cations, Divalent; Cell Line; Chlorides; Colon; Cyclic AMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diffusion; Humans; Membrane Proteins; Oligopeptides; Peptide Fragments; Peptides

1992