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 40 for “"cystic fibrosis transmembrane conductance regulator"”.
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The Cystic Fibrosis Transmembrane Conductance Regulator: Regulation by HSP-90
<p>The hypothesis of this project is that a functionally relevant association exists between CFTR and Hsp90. In testing this hypothesis, (a) the association of CFTR and Hsp90 is confirmed via crosslinking and immunoprecipitation of CFTR from stably transfected HEK-293 cells, electrophoretic …
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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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Defining a role for the cystic fibrosis transmembrane conductance regulator in the heart
While cystic fibrosis (CF) is commonly thought of as a lung disease, since it’s first description 70 years ago we have come to understand that loss of CFTR (cystic fibrosis transmembrane conductance regulator) function affects numerous tissues and systems throughout the body. Concerning the heart, …
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Structural Study of Disease Relevant ABC Transporters-Cystic Fibrosis Transmembrane Conductance Regulator and ABCA4
… ABC transporters consist of four subunits— two transmembrane domains (TMDs) which form the substrate transport pathway and two nucleotide binding domains (NBDs) which dimerize upon ATP binding to provide energy for substrate transport. Most mammalian ABC transporters are exporters, with three …
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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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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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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 CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Channel: Anion Permeation, and Regulation by Adenylyl Cyclase and ATP Hydrolysis
… Using whole-cell CFTR Cl<sup>-</sup> conductance as an on-line assay of cAMP levels, we examined the interaction of the adenylyl cyclase (AC) modulators forskolin (Fsk), and the GTP-binding proteins G<sub>s</sub> and G<sub>i</sub>. In the presence of GTP, maximal activation of …
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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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Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapies in Cystic Fibrosis: A Retrospective Evaluation of a Nationwide Specialty Pharmacy Database
<p>Background: Cystic fibrosis (CF) is a progressive, genetic disorder caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Patients with CF experience excessive mucus build up and infections leading to complications in the pancreas, lungs, and other organs. …
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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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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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