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10.1172/jci.insight.88730 http://scihub22266oqcxt.onion/10.1172/jci.insight.88730 C5027966!5027966!27656681     free     free     free Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       JCI+Insight ä ; 1 (14): ä Nephropedia Template TP {{tp|p=27656681|t=ä. Lentiviral-mediated phenotypic correction of cystic fibrosis pigs |pdf=|usr=}}{{27656681}} gab.com Text Lentiviral-mediated phenotypic correction of cystic fibrosis pigs JO: JCI Insight http://www.kidney.de/pget.php?&tw=1&pmid=27656681 #FOAvip Cystic Fibrosis (CF) is an autosomal recessive disease caused by mutations in CF transmembrane conductance regulator (CFTR), resulting in defective anion transport. Regardless of the disease-causing mutation, gene therapy is a strategy to restore anion transport to airway epithelia. Indeed, viral vector?delivered CFTR can complement the anion channel defect. In this proof-of-principle study, functional in vivo CFTR channel activity was restored in the airways of CF pigs using a feline immunodeficiency virus?based (FIV-based) lentiviral vector pseudotyped with the GP64 envelope. Three newborn CF pigs received aerosolized FIV-CFTR to the nose and lung. Two weeks after viral vector delivery, epithelial tissues were analyzed for functional correction. In freshly excised tracheal and bronchus tissues and cultured ethmoid sinus cells, we observed a significant increase in transepithelial cAMP-stimulated current, evidence of functional CFTR. In addition, we observed increases in tracheal airway surface liquid pH and bacterial killing in CFTR vector?treated animals. Together, these data provide the first evidence to our knowledge that lentiviral delivery of CFTR can partially correct the anion channel defect in a large-animal CF model and validate a translational strategy to treat or prevent CF lung disease. Twit Text FOAVip Lentiviral-mediated phenotypic correction of cystic fibrosis pigs ????JCI Insight http://www.kidney.de/pget.php?&tw=1&pmid=27656681 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=27656681 #FOAvip English Wikipedia <ref>{{Cite pmid|27656681}}</ref> Lentiviral-mediated phenotypic correction of cystic fibrosis pigs #MMPMID27656681 Cooney AL; Abou Alaiwa MH; Shah VS; Bouzek DC; Stroik MR; Powers LS; Gansemer ND; Meyerholz DK; Welsh MJ; Stoltz DA; Sinn PL; McCray PB JCI Insight ä[]; 1 (14): ä PMID27656681show ga Cystic Fibrosis (CF) is an autosomal recessive disease caused by mutations in CF transmembrane conductance regulator (CFTR), resulting in defective anion transport. Regardless of the disease-causing mutation, gene therapy is a strategy to restore anion transport to airway epithelia. Indeed, viral vector?delivered CFTR can complement the anion channel defect. In this proof-of-principle study, functional in vivo CFTR channel activity was restored in the airways of CF pigs using a feline immunodeficiency virus?based (FIV-based) lentiviral vector pseudotyped with the GP64 envelope. Three newborn CF pigs received aerosolized FIV-CFTR to the nose and lung. Two weeks after viral vector delivery, epithelial tissues were analyzed for functional correction. In freshly excised tracheal and bronchus tissues and cultured ethmoid sinus cells, we observed a significant increase in transepithelial cAMP-stimulated current, evidence of functional CFTR. In addition, we observed increases in tracheal airway surface liquid pH and bacterial killing in CFTR vector?treated animals. Together, these data provide the first evidence to our knowledge that lentiviral delivery of CFTR can partially correct the anion channel defect in a large-animal CF model and validate a translational strategy to treat or prevent CF lung disease. äLentiviral-mediated phenotypic correction of cystic fibrosis pigs (epmc).pdf DeepDyve Pubget Overpricing