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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 33 for “"Cystic fibrosis transmembrane conductance regulator (CFTR)"”.
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The Expression and Characterization of Human Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Tobacco
The cystic fibrosis transmembrane conductance regulator (CFTR) is one of the most studied membrane protein models because of its clear clinical significance. Mutations within the CFTR gene lead to cystic fibrosis, the most common autosomal recessive genetic disorder in the Caucasian population. …
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Ionentransportstörungen im Rektumepithel von Mukoviszidosepatienten - funktionelle Charakterisierung und Bedeutung für die Diagnostik
… als krankheitsverusachend geltenden Defekt des "cystic fibrosis transmembrane conductance regulator (CFTR)" funktionell nachzuweisen.
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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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Physical and Functional Coupling of CFTR and PDE3A
… is physically and functionally coupled to cystic fibrosis transmembrane conductance regulator (CFTR). PDE3A inhibition increases cyclic adenosine 3′, 5′-monophosphate (cAMP) levels in a compartmentalized manner at the plasma membrane, which potentiates CFTR channel function and further …
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Macromolecular Complexes of Cystic Fibrosis Transmembrane Conductance Regulator Alter Fluid Transport in inflammatory Bowel Disorders
<p>Macromolecular complexes of cystic fibrosis transmembrane conductance regulator (CFTR) comprise of network of proteins that can regulate cAMP-/cGMP-activated CFTR chloride channel function. We report the physical and functional coupling of CFTR with nitric oxide (NO) producing enzyme-inducible …
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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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Untersuchung der durch den Transkriptionsfaktor NF-kB vermittelten ER-Overload Response bei Zystischer Fibrose
… Zystischen Fibrose (CF) sind Mutationen eines Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) genannten Gens, das für einen Chloridkanal kodiert. Die am häufigsten vorkommende Mutation, DF508-CFTR, bildet ein mutiertes Protein, das im Gegensatz zur Wildtyp-Form im Endoplasmatischen …
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Cystic fibrosis gene repair: correction of ΔF508 using ZFN and CRISPR/Cas9 guide RNA gene editing tools
Cystic Fibrosis (CF) is an autosomal recessive monogenic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene with the ΔF508 mutation accounting for approximately 70% of all CF cases worldwide. This thesis investigates whether existing zinc finger …
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Candidate genes other than the CFTR gene as possible modifiers of pulmonary disease severity in cystic fibrosis
Cystic fibrosis (CF) is a single gene Mendelian disorder characterized by pulmonary disease and pancreatic insufficiency. Pulmonary disease is the major cause of death in CF patients. Although some cystic fibrosis transmembrane conductance regulator (CFTR) genotypes are associated with less severe …
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Cystic fibrosis and the gut microbiota
… be altered in numerous disease states, including Cystic Fibrosis (CF) cohorts. This thesis investigates the gut microbiota and microbiome in CF persons and how it may be manipulated through interventions with the potential to improve respiratory symptoms in CF persons. Firstly, in order to survey …
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The CFTR Folding Pathway: Implications for the Identification and Development of CF Therapeutics
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein is a member of the ABC transporter superfamily, important for Cl- conductance at the apical cell membrane. Loss-of-function of CFTR leads to Cystic Fibrosis (CF), a fatal genetic disease affecting 70,000 people worldwide. There …
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DEVELOPMENT AND TESTING OF A FLEXIBLE PLASTIC HYDROGEN ION-SELECTIVE ELECTRODE FOR IN VIVO pH MEASUREMENTS OF AIRWAY SURFACE LIQUID
Cystic fibrosis (CF) is the most common fatal autosomal recessive genetic disorder among people of Caucasian ancestry. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a transepithelial transporter responsible for ion transport and …
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Einfluss der intrazellulären Cl- Konzentration auf den epithelialen Na+Kanal
… (CF) deutlich. Aufgrund von Mutationen im Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) kann ENaC nicht mehr inhibiert werden. In den von CF betroffenen Epithelien liegt daher neben einer verminderten Cl--Leitfähigkeit eine erhöhte Na+-Leitfähigkeit vor. Ziel der vorliegenden …
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Synthesis Of Non-Natural Fmoc-Protected Amino Acids To Provide Novel Fluorescent Anion Probes And Their Incorporation Into Synthetic Peptides
Cystic fibrosis is a genetic disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR serves to control the gradient of chloride and bicarbonate ions across the cellular membrane of epithelial tissues (e.g., mucosa, intestinal walls, and lungs). …
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Cotranslational Folding of CFTR
The life of the cystic fibrosis transmembrane conductance regulator (CFTR) protein in the cell is dictated by its biogenesis, cellular trafficking, regulated function, and destruction. Cystic fibrosis (CF) is the direct result of perturbations in these processes. Treatment of CF mandates the …
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Structure-Function Studies on Human Epithelial Cystic Fibrosis Chloride Channels Expressed in Xenopus Laevis Oocytes
<p>The cystic fibrosis transmembrane conductance regulator (CFTR) Cl<sup>-</sup> channel is an ATP-binding cassette protein, comprising two transmembrane domains, two nucleotide binding domains (NBD1, NBD2) and a regulatory (R) domain. Channel gating is controlled by R-domain phosphorylation and by …
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Regulation of airway epithelial cell migration by the cystic fibrosis transmembrane conductance regulator.
… essential for cilia movement. The ion channel cystic fibrosis transmembrane conductance regulator (CFTR), transports Cl- and HCO3 - in the airways and other tissues, and controls the depth of the periciliary liquid layer. Cystic fibrosis is a fatal genetic disease in which CFTR is …
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The novel role of epidermal growth factor (EGF) in the regulation of ion channels in the calu-3 submucosal cell line
Cystic fibrosis transmembrane conductance regulator (CFTR) is a cell membrane bound chloride ion channel regulated by cyclic AMP-dependent phosphorylation and levels of intracellular ATP. Mutations in this channel, such as the common deletion of phenylalanine at residue 508 (CFTRΔF508), leads to a …
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Characterisation of Guanylin and Uroguanylin in the Digestive System
… A (PKA). Both PKGII and PKA phosphorylate the cystic fibrosis transmembrane conductance regulator (CFTR), leading to an efflux of chloride in the lumen, resulting in fluid secretion into the intestine. The importance of this hormone-receptor system in human gastrointestinal physiology is …
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Single-Molecule Studies of CFTR Gating and Pharmacology
<p>The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel expressed in the apical membrane of epithelial tissues. Alterations in CFTR that disrupt activity cause cystic fibrosis, a fatal disease which is characterized by systemic salt and fluid dysregulation. By …
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