Found 17 datasets matching "cystic-fibrosis".
-
The identification of the cystic fibrosis (CF) gene opened the way for gene therapy. In the ten years since then, proof of principle in vitro and then in animal models in vivo has been followed by...
Search relevance: 276.65 | Views last month: 2 -
The cystic fibrosis transmembrane conductance regulator (CFTR) has been known for the past 11 years to be a membrane glycoprotein with chloride channel activity. Only recently has the...
Search relevance: 276.61 | Views last month: 1 -
The increase in numbers of adults with cystic fibrosis (CF) has allowed us to identify previously unrecognized chronic complications of CF, as well as appreciate unique presentations of cystic...
Search relevance: 274.80 | Views last month: 0 -
Cystic fibrosis is a genetic disease that is associated with abnormal sweat electrolytes, sino-pulmonary disease, exocrine pancreatic insufficiency, and male infertility. Insights into...
Search relevance: 259.07 | Views last month: 1 -
Background Endoplasmic reticulum retention of misfolded cystic fibrosis transmembrane conductance regulator (CFTR) mutants and their rapid degradation is the major cause of cystic...
Search relevance: 250.88 | Views last month: 0 -
Background Attempts at gene therapy for the pulmonary manifestations of Cystic Fibrosis have relied mainly on airway delivery. However the efficiency of gene transfer and expression in...
Search relevance: 242.68 | Views last month: 7 -
Background Excessive loss of bile acids in stool has been reported in patients with cystic fibrosis. Some data suggest that a defect in mucosal bile acid transport may be the mechanism...
Search relevance: 133.14 | Views last month: 0 -
Secretory leukocyte proteinase inhibitor and elafin are two low-molecular-mass elastase inhibitors that are mainly synthesized locally at mucosal sites. It is thought that their physicochemical...
Search relevance: 122.96 | Views last month: 0 -
Aim To demonstrate the effects of combined inhaled nitric oxide and surfactant replacement as treatment for acute respiratory distress syndrome. This treatment has not previously been...
Search relevance: 122.27 | Views last month: 0 -
Recombinant adeno-associated virus (rAAV) vectors mediate long-term gene transfer without any known toxicity. The primary limitation of rAAV has been the small size of the virion (20 nm), which...
Search relevance: 121.08 | Views last month: 0 -
Background SFHR (small fragment homologous replacement)-mediated targeting is a process that has been used to correct specific mutations in mammalian cells. This process involves both...
Search relevance: 112.23 | Views last month: 0 -
Pulmonary surfactant is a surface active material composed of both lipids and proteins that is produced by alveolar type II pneumocytes. Abnormalities of surfactant in the immature lung or in the...
Search relevance: 77.88 | Views last month: 2 -
These datasets contain summary data about the Annual California Children’s Services (CCS) Whole Child Model (WCM) program. These summary files are intended to accompany the CCS Power BI Dashboard...
Search relevance: 68.86 | Views last month: 7 -
Background This study describes the functional interaction between the putative Ca2+ channel TRP4 and the cystic fibrosis transmembrane conductance regulator, CFTR, in mouse aorta...
Search relevance: 67.88 | Views last month: 0 -
Regeneration and restoration of the airway epithelium after mechanical, viral or bacterial injury have a determinant role in the evolution of numerous respiratory diseases such as chronic...
Search relevance: 66.28 | Views last month: 3 -
These secondary data have been produced from the gene expression data generated from duodena of mice exposed to hexavalent chromium in drinking water and deposited in the GEO repository under...
Search relevance: 63.10 | Views last month: 2 -
Background Pseudomonas aeruginosa and Burkholderia cepacia infections of cystic fibrosis patients' lungs are often resistant to conventional antibiotic therapy. Protegrins are...
Search relevance: 48.80 | Views last month: 0
