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Differential effects of ELX/TEZ/IVA on organ-specific CFTR function in two patients with the rare CFTR splice mutations c.273+1G>A and c.165-2A>G.
- Published in:
- Frontiers in Pharmacology, 2023, v. 14, p. 1, doi. 10.3389/fphar.2023.1153656
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- Article
Post-approval studies with the CFTR modulators Elexacaftor-Tezacaftor—Ivacaftor.
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- Frontiers in Pharmacology, 2023, v. 14, p. 1, doi. 10.3389/fphar.2023.1158207
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- Publication type:
- Article
Changes in cystic fibrosis transmembrane conductance regulator protein expression prior to and during elexacaftor-tezacaftor-ivacaftor therapy.
- Published in:
- Frontiers in Pharmacology, 2023, v. 14, p. 1, doi. 10.3389/fphar.2023.1114584
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- Publication type:
- Article
Metabolite profiling of the cold adaptation of Pseudomonas putida KT2440 and cold‐sensitive mutants.
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- Environmental Microbiology Reports, 2019, v. 11, n. 6, p. 777, doi. 10.1111/1758-2229.12793
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- Publication type:
- Article
Intraclonal genome diversity of the major Pseudomonas aeruginosa clones C and PA14.
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- Environmental Microbiology Reports, 2016, v. 8, n. 2, p. 227, doi. 10.1111/1758-2229.12372
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- Article
Habitat-associated skew of clone abundance in the Pseudomonas aeruginosa population.
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- Environmental Microbiology Reports, 2015, v. 7, n. 6, p. 955, doi. 10.1111/1758-2229.12340
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- Publication type:
- Article
Mild course of cystic fibrosis associated with heterozygosity for infrequent mutations in the first nucleotide-binding fold of CFTR.
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- 1992
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- Publication type:
- journal article
Comparative genomics of green sulfur bacteria.
- Published in:
- Photosynthesis Research, 2010, v. 104, n. 2/3, p. 137, doi. 10.1007/s11120-009-9515-2
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- Article
Virus-Induced Type I Interferon Deteriorates Control of Systemic Pseudomonas Aeruginosa Infection.
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- Cellular Physiology & Biochemistry (Karger AG), 2015, v. 36, n. 6, p. 2379, doi. 10.1159/000430200
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- Article
Immunochemical Analysis of Mutant CFTR in Lung Explants.
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- Cellular Physiology & Biochemistry (Karger AG), 2012, v. 30, n. 3, p. 587, doi. 10.1159/000341440
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- Article
Effects of the Serine/Threonine Kinase SGK1 on the Epithelial Na<sup>+</sup> Channel (ENaC) and CFTR: Implications for Cystic Fibrosis.
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- Cellular Physiology & Biochemistry (Karger AG), 2001, v. 11, n. 4, p. 209, doi. 10.1159/000051935
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- Article
Competitive fitness of Pseudomonas aeruginosa isolates in human and murine precision-cut lung slices.
- Published in:
- Frontiers in Cellular & Infection Microbiology, 2022, v. 12, p. 1, doi. 10.3389/fcimb.2022.992214
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- Publication type:
- Article
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis.
- Published in:
- Nature Medicine, 2008, v. 14, n. 4, p. 382, doi. 10.1038/nm1748
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- Article
Epidemiology Of Chronic Pseudomonas aeruginosa Infections In Cystic Fibrosis.
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- Journal of Infectious Diseases, 1994, v. 170, n. 6, p. 1616, doi. 10.1093/infdis/170.6.1616
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- Publication type:
- Article
Feasibility and Variability of Measuring the Lung Clearance Index in a Multi-Center Setting.
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- Pediatric Pulmonology, 2012, v. 47, n. 7, p. 649, doi. 10.1002/ppul.21610
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- Publication type:
- Article
Head-Out Spirometry Accurately Monitors the Course of Pseudomonas aeruginosa Lung Infection in Mice.
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- Respiration, 2010, v. 80, n. 4, p. 340, doi. 10.1159/000319442
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- Article
A regulatory SNP modifies cystic fibrosis by disrupting NFκB complex binding on FAS.
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- 2015
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- Publication type:
- Abstract
Multiple roles of Pseudomonas aeruginosa TBCF10839 PilY1 in motility, transport and infection.
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- Molecular Microbiology, 2009, v. 71, n. 3, p. 730, doi. 10.1111/j.1365-2958.2008.06559.x
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- Publication type:
- Article
Molecular Epidemiology of Chronic Pseudomonas aeruginosa Airway Infections in Cystic Fibrosis.
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- PLoS ONE, 2012, v. 7, n. 11, p. 1, doi. 10.1371/journal.pone.0050731
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- Publication type:
- Article
A New Role of the Complement System: C3 Provides Protection in a Mouse Model of Lung Infection with Intracellular Chlamydia psittaci.
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- PLoS ONE, 2012, v. 7, n. 11, p. 1, doi. 10.1371/journal.pone.0050327
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- Publication type:
- Article
Genometa - A Fast and Accurate Classifier for Short Metagenomic Shotgun Reads.
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- PLoS ONE, 2012, v. 7, n. 8, p. 1, doi. 10.1371/journal.pone.0041224
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- Publication type:
- Article
In-Vivo Expression Profiling of Pseudomonas aeruginosa Infections Reveals Niche-Specific and Strain- Independent Transcriptional Programs.
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- PLoS ONE, 2011, v. 6, n. 9, p. 1, doi. 10.1371/journal.pone.0024235
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- Publication type:
- Article
Abundant Oligonucleotides Common to Most Bacteria.
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- PLoS ONE, 2010, v. 5, n. 3, p. 1, doi. 10.1371/journal.pone.0009841
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- Article
Fitness of Isogenic Colony Morphology Variants of Pseudomonas aeruginosa in Murine Airway Infection.
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- PLoS ONE, 2008, v. 3, n. 2, p. 1, doi. 10.1371/journal.pone.0001685
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- Publication type:
- Article
The Neglected Intrinsic Resistome of Bacterial Pathogens.
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- PLoS ONE, 2008, v. 3, n. 2, p. 1, doi. 10.1371/journal.pone.0001619
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- Publication type:
- Article
Pseudomonas aeruginosa Population Biology in Chronic Obstructive Pulmonary Disease.
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- Journal of Infectious Diseases, 2009, v. 200, n. 12, p. 1928, doi. 10.1086/648404
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- Publication type:
- Article
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two Alleles.
- Published in:
- American Journal of Respiratory & Critical Care Medicine, 2022, v. 206, n. 3, p. 311, doi. 10.1164/rccm.202201-0219OC
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- Publication type:
- Article
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two Alleles.
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- 2022
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- Publication type:
- journal article
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles.
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- American Journal of Respiratory & Critical Care Medicine, 2022, v. 205, n. 5, p. E1, doi. 10.1164/rccm.202110-2249oc
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- Publication type:
- Article
Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis.
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- 2018
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- Publication type:
- journal article
Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor.
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- 2015
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- Publication type:
- Letter
Acute intratracheal Pseudomonas aeruginosa infection in cystic fibrosis mice is age-independent.
- Published in:
- Respiratory Research, 2011, v. 12, n. 11, p. 1, doi. 10.1186/1465-9921-12-148
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- Publication type:
- Article
Acute intratracheal Pseudomonas aeruginosa infection in cystic fibrosis mice is age-independent.
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- 2011
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- Publication type:
- journal article
Comparative genomics and biological characterization of sequential Pseudomonas aeruginosa isolates from persistent airways infection.
- Published in:
- BMC Genomics, 2015, v. 16, p. 1, doi. 10.1186/s12864-015-2276-8
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- Publication type:
- Article
Long-term tobramycin aerosol therapy in cystic fibrosis.
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- Pediatric Pulmonology, 1989, v. 6, n. 2, p. 91, doi. 10.1002/ppul.1950060207
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- Publication type:
- Article
Role of CD95 in pulmonary inflammation and infection in cystic fibrosis.
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- Journal of Molecular Medicine, 2012, v. 90, n. 9, p. 1011, doi. 10.1007/s00109-012-0867-2
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- Publication type:
- Article
Hierarchical fine mapping of the cystic fibrosis modifier locus on 19q13 identifies an association with two elements near the genes CEACAM3 and CEACAM6.
- Published in:
- Human Genetics, 2010, v. 127, n. 4, p. 383, doi. 10.1007/s00439-009-0779-6
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- Article
The TNFα receptor TNFRSF1A and genes encoding the amiloride-sensitive sodium channel ENaC as modulators in cystic fibrosis.
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- Human Genetics, 2006, v. 119, n. 3, p. 331, doi. 10.1007/s00439-006-0140-2
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- Publication type:
- Article
TheCLCAgene locus as a modulator of the gastrointestinal basic defect in cystic fibrosis.
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- Human Genetics, 2004, v. 115, n. 6, p. 483, doi. 10.1007/s00439-004-1190-y
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- Publication type:
- Article
Genes in the vicinity of CFTR modulate the cystic fibrosis phenotype in highly concordant or discordant F508del homozygous sib pairs.
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- Human Genetics, 2003, v. 112, n. 1, p. 1, doi. 10.1007/s00439-002-0839-7
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- Publication type:
- Article
Characterization of a novel 21-kb deletion, CFTRdele2,3(21 kb), in the CFTR gene: a cystic fibrosis mutation of Slavic origin common in Central and East Europe.
- Published in:
- Human Genetics, 2000, v. 106, n. 3, p. 259, doi. 10.1007/s004390000246
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- Publication type:
- Article
Structural genome variants of Pseudomonas aeruginosa clone C and PA14 strains.
- Published in:
- Frontiers in Microbiology, 2023, v. 14, p. 1, doi. 10.3389/fmicb.2023.1095928
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- Publication type:
- Article
Mucin adhesion of serial cystic fibrosis airways Pseudomonas aeruginosa isolates.
- Published in:
- Frontiers in Cellular & Infection Microbiology, 2024, p. 1, doi. 10.3389/fcimb.2024.1448104
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- Publication type:
- Article
The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells.
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- European Journal of Human Genetics, 2014, v. 22, n. 5, p. 660, doi. 10.1038/ejhg.2013.209
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- Publication type:
- Article
Clinical and molecular characterization of the potential CF disease modifier syntaxin 1A.
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- European Journal of Human Genetics, 2013, v. 21, n. 12, p. 1462, doi. 10.1038/ejhg.2013.57
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- Publication type:
- Article
CLCA4 variants determine the manifestation of the cystic fibrosis basic defect in the intestine.
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- European Journal of Human Genetics, 2013, v. 21, n. 6, p. 691, doi. 10.1038/ejhg.2012.234
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- Publication type:
- Article
Initial interrogation, confirmation and fine mapping of modifying genes: STAT3, IL1B and IFNGR1 determine cystic fibrosis disease manifestation.
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- European Journal of Human Genetics, 2011, v. 19, n. 12, p. 1281, doi. 10.1038/ejhg.2011.129
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- Publication type:
- Article
Differential decay of parent-of-origin-specific genomic sharing in cystic fibrosis-affected sib pairs maps a paternally imprinted locus to 7q34.
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- European Journal of Human Genetics, 2010, v. 18, n. 5, p. 553, doi. 10.1038/ejhg.2009.229
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- Publication type:
- Article
Transmission ratio distortion and maternal effects confound the analysis of modulators of cystic fibrosis disease severity on 19q13.
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- European Journal of Human Genetics, 2007, v. 15, n. 7, p. 774, doi. 10.1038/sj.ejhg.5201825
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- Publication type:
- Article
Long-Term Microevolution of Pseudomonas aeruginosa Differs between Mildly and Severely Affected Cystic Fibrosis Lungs.
- Published in:
- American Journal of Respiratory Cell & Molecular Biology, 2018, v. 59, n. 2, p. 246, doi. 10.1165/rcmb.2017-0356OC
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- Publication type:
- Article