Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 20 of 105 for “"Cystic fibrosis (CF)"”.
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Real-world Orkambi Cork (ROCK) study - a prospective 12 months analysis addressing the impact of CFTR modulation in patients with cystic fibrosis homozygous for F508del CFTR variant
Cystic fibrosis (CF) stands as one of modern medicine's success stories, with significant and sustained improvements in survival rates, transforming it from a childhood fatal condition to one of adult survival. Historically, CF management has centered on addressing the consequences of CFTR …
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Towards gene therapy for cystic fibrosis: enhanced green fluorescent protein as a reporter of promoter activity
Cystic Fibrosis (CF) is the most common lethal inherited disease, affecting -1/2000 live births. Although the genetic lesion, a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, has been elucidated, the exact mechanism whereby this causes the debilitating disease …
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Novel agents with inhibitory activity against the Burkholderia cepacia complex
… account for 95% of morbidity and mortality in cystic fibrosis (CF) patients, and include a limited spectrum of bacteria; Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, and members of the Burkholderia cepacia complex (Bcc). "B. cepacia" was first recognised in the late …
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Designing a Reactive Warhead to Bind and Inhibit Pseudomonas aeruginosa’s Periplasmic Protein, Inhibitor of Vertebrate Lysozyme
… patients such as those suffering from cystic fibrosis (CF). Due to the emerging antibiotic resistance of <em>P. aeruginosa</em>, it is becoming increasingly more challenging to treat an infection by traditional means. Further complicating treatment, <em>P. aeruginosa</em> secretes a …
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AIRWAY SURFACE HYPERVISCOSITY AND DEFECTIVE MUCOCILIARY TRANSPORT BY IL-17/TNF-ALPHA ARE CORRECTED BY BETA-ADRENERGIC STIMULUS
… activity of ion channels and transporters. In cystic fibrosis (CF), loss of CFTR chloride channel function causes airway surface dehydration, bacterial infection, and inflammation. We investigated the effects of IL-17A plus TNF-α, 2 cytokines with relevant roles in CF and other chronic lung …
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Immune Dysfunction in Cystic Fibrosis
Cystic Fibrosis (CF) is one of the most widespread life-shortening genetic diseases. CF is often diagnosed at birth; there is no cure, and many CF patients die from chronic lung disease at a young age. Patients with CF experience declining pulmonary function related to chronic airway infection, …
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Sequence-Specific Gene Correction of Cystic Fibrosis Airway Basal Cells
<p>Cystic fibrosis (CF) is a lethal monogenic disease resulting from mutations in the <em>CFTR </em>gene which encodes a protein involved in regulating anion trans-epithelial transport. A three-base deletion in <em>CFTR </em>(termed as ΔF508 mutation), wherein CFTR protein is misfolded leading to …
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Hispanic Mothers’ Narratives: Experiences of Parenting a Child with Cystic Fibrosis, Health Beliefs, and Health Care Experiences Related to Cystic Fibrosis
… the United States and is also a minority in the cystic fibrosis (CF) community. Therefore, an increase in CF in the U.S. might be anticipated. This study explored stories of Hispanic mothers’ perspectives of parenting their child with CF, health beliefs, and health care experiences. The study …
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New gating states of Cystic Fibrosis transmembrane conductance regulator discovered via studying pathogenic mutations, pharmacological reagents and ATP analogs.
… THE UNIVERSITY OF MISSOURI AT AUTHOR'S REQUEST.] Cystic Fibrosis (CF), caused by the loss of function in the gene for the protein cystic fibrosis transmembrane conductance (CFTR), is one of the most common lethal genetic diseases in the United States. By studying the structural/functional …
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The experience of transferring to adult health care for adolescents with cystic fibrosis
… treatment and management, adolescents with cystic fibrosis (CF) now have a longer life expectancy. Consequently, more adolescents will be transferred from paediatric to adult health care. Currently, transfer to adult health care takes place on an adhoc basis. Little research however, is …
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Development and Characterisation of Solid-State Ion-Selective Electrodes, and their Application to Sweat Analysis for Cystic Fibrosis Diagnosis
Cystic Fibrosis (CF) is one of the most common genetic diseases affecting Caucasians that leads to early deathi. It is Ireland’s most common life- threatening inherited disease. Early diagnosis facilitates early implementation of therapy, which can significantly improve the prognosis and life …
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Effect of Exopolysaccharides on Pseudomonas aeruginosa Physiology
… produced and co-localize with P. aeruginosa in cystic fibrosis (CF) lung infections. I also demonstrate that ionic interactions between Pel and extracellular DNA (eDNA) increase tolerance to aminoglycoside treatment. Additionally, I determine that cell-association of Psl is promoted …
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The cystic fibrosis transmembrane conductance regulator and acid-base transporters of the murine duodenum
… involves the apical membrane activities of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl⁻ channel, the protein that is defective in cystic fibrosis (CF), and Cl⁻/HCO₃⁻ exchangers. Under basal conditions, studies of CF patients and mouse models indicate that HCO₃⁻ secretion by …
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Modulation of airway mucin glycosylation by Pseudomonas aeruginosa pyocyanin
Cystic fibrosis (CF) patients battle life-long pulmonary infections with the respiratory pathogen Pseudomonas aeruginosa (PA). A favorable niche for PA growth is provided by an overabundance of mucus in CF airways. When compared to that of non-CF individuals, mucus of CF airways has been found to …
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Amphotericin B ion channels restore cystic fibrosis airway surface physiology
… reduced or absent protein production, such as cystic fibrosis (CF). Amphotericin B (AmB) is a natural product known to self-assemble into nonspecific ion channels in sterol-containing lipid membranes. It was long believed that AmB killed yeast through its inherent ion channel activity, existing …
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Highly Saturated Transposon Sequencing identifies genes impacting Staphylococcus aureus pathogenesis in macrophages
… airways of chronically infected individuals with cystic fibrosis (CF) revealed significantly greater rates of in vivo selection in candidate genes than factors not associated with macrophage invasion. This study implicates a core set of genes necessary to support macrophage invasion by S. aureus, …
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Correction of Cystic Fibrosis-Specific Induced Pluripotent Stem Cells
<p>Cystic Fibrosis (CF), affecting 1 in 3,500 live births in the US, is a disease caused by aberrant expression of the Cystic Fibrosis Transmemebrane Conductance Regulator (CFTR). While a multi-organ disease, CF-related complications and degradation of the lung is the leading cause of mortality. …
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Self-Reported Oral health Assessment and Attitudes Toward Oral Health for Adults With Cystic Fibrosis
<p>Purpose: Cystic fibrosis (CF) is a chronic genetic disorder that an individual has at birth. Cystic fibrosis impacts equally all races, gender and becomes worse over time. The aim of this current study was to evaluate oral health status, interaction with the oral health system, and oral health …
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Physiological and perceptual responses during self-regulated exercise in children with cystic fibrosis
… during self-regulated exercise in children with cystic fibrosis (CF). Methods: Twelve children (10.8 ± 2.0 y) with CF made 3 separate visits to the research laboratory in the school of Health and Human Performance in DCU. The first visit was used to determine VO2peak and to anchor the children’s …
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Cystic fibrosis in black African children in South Africa: a case control study
Background Cystic fibrosis (CF) is described more commonly in Caucasian populations in whom p.Phe508del is the most common mutation. There is a paucity of data of CF in black African children. The aim of this study was to describe and compare the presentation and outcomes of black African children …
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