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Showing 1 to 20 of 77 for “"CFTR"”.
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Cotranslational Folding of CFTR
… 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 understanding of the …
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CFTR klórgöng í ristilþekju hænsnfugla (Gallus gallus)
… huldu. Cystic fibrosis transmembrane regulator (CFTR) eru ein gerð af klórgöngum sem eru algeng í þekjuvef. Þau þjóna meðal annars stóru hlutverki í ristilþekju spendýra. Því var tilgangurinn með þessari rannsókn að kanna tilvist CFTR í ristilþekju hænsnfugla. Framkvæmd: Ristill hænsna, frá …
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Physical and Functional Coupling of CFTR and PDE3A
… 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 clusters PDE3A and CFTR into microdomains. Actin skeleton …
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Single-Molecule Studies of CFTR Gating and Pharmacology
… 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 contrast, …
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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
… has centered on addressing the consequences of CFTR dysfunction. The introduction of Lumacaftor-Ivacaftor marks a pivotal shift, allowing researchers to assess the impact of CFTR function restoration on both pulmonary disease and extrapulmonary manifestations. Globally, approximately 82% of …
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Quantitative Bestimmung der 9-minus-Splice-Varante in CFTR-mRNA aus Rektumschleimhautbiopsie und deren Abhängigkeit von der Länge des Poly-T-Traktes in Intron 8 des CFTR-Gens
… des TGmTn-Polymorphismus in Intron 8 des CFTR-Gens auf das Fehlspleißen des Exon 9 der CFTR-mRNA in Rektummukosabiopsien von 26 Probanden untersucht. Dem ermittelten Genotyp wurde der mittels neu etablierter semiquantitativer RT-PCR bestimmte Anteil an korrekt (9-Plus) und fehlgespleißter …
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The CFTR Folding Pathway: Implications for the Identification and Development of CF Therapeutics
… 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 are …
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Role of the Lipid Environment and Cholesterol Content in the Function of CFTR Modulators
… Fibrosis Transmembrane Conductance Regulator (CFTR). Despite CFTR being a protein located in epithelial cell membranes, it has not been established whether the membrane surrounding CFTR plays a role in the function of the recently-discovered CFTR modulators. In concert with a variety of …
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The Expression and Characterization of Human Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Tobacco
… 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. CFTR, a …
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Studio in vitro dell'espressione di varianti del gene CFTR con ruolo patofisiologico non ancora definito
The mutational screening of the CFTR gene, performed with three progressive steps (screening of the most common mutations with a commercial diagnostic kit, analysis of deletions/duplications with MLPA assay and sequencing of the coding CFTR regions) leads to a detection rate of about 94%. The …
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Candidate genes other than the CFTR gene as possible modifiers of pulmonary disease severity in cystic fibrosis
… fibrosis transmembrane conductance regulator (CFTR) genotypes are associated with less severe disease, patients possessing the same genotype show great variation in pulmonary disease severity and progression. Genes involved in modulating the inflammatory response and genes increasing …
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The Cystic Fibrosis Transmembrane Conductance Regulator: Regulation by HSP-90
… 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 separation of cross-linked proteins, and protein …
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The CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) Channel: Anion Permeation, and Regulation by Adenylyl Cyclase and ATP Hydrolysis
<p>CFTR Cl<sup>-</sup> channel function and regulation were studied in guinea-pig ventricular myocytes, using either the whole-cell or excised inside-out patch configurations of the patch clamp technique. A typical myocyte cell membrane contained a total of ~1400 CFTR channels; channel density was …
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LIPID-BASED APPROACHES TO OPTIMIZE THE EFFECTIVENESS OF INNOVATIVE DRUG MODULATORS IN CYSTIC FIBROSIS
… Fibrosis Transmembrane Conductance Regulator (CFTR) gene. This gene encodes a chloride-conducting transmembrane channel crucial for ion transport across epithelial cells. CFTR mutations lead to impaired expression and function of the channel, causing disrupted chloride ion transport in …
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Influencia de la brecha de sexo y el genotipo CFTR sobre la debilidad muscular en adultos con fibrosis quística.
… transmembrana de la fibrosis quística (CFTR). Los pacientes que sufren esta enfermedad se caracterizan por tener una función muscular periférica deficiente. Esta alteración muscular repercute negativamente en la capacidad para generar fuerza y; por lo tanto, en la funcionalidad durante …
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Structural Study of Disease Relevant ABC Transporters-Cystic Fibrosis Transmembrane Conductance Regulator and ABCA4
… fibrosis transmembrane conductance regulator (CFTR)—an chloride channel and ABCA4 (aka the Rim protein and ABCR)—an retinylidene-PE importer. In addition to their unique functional properties, both CFTR and ABCA4 are very important in human health. Mutations in CFTR cause cystic fibrosis, a …
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Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapies in Cystic Fibrosis: A Retrospective Evaluation of a Nationwide Specialty Pharmacy Database
… 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. There has been a paradigm shift in the treatment of CF with the introduction of CFTR modulator …
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Defining a role for the cystic fibrosis transmembrane conductance regulator in the heart
… 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, right ventricular dysfunction secondary to pulmonary abnormalities has long been recognized. However, …
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