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213 results

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.
Van Goor F, Hadida S, Grootenhuis PD, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu PA. Van Goor F, et al. Among authors: frizzell ra. Proc Natl Acad Sci U S A. 2011 Nov 15;108(46):18843-8. doi: 10.1073/pnas.1105787108. Epub 2011 Oct 5. Proc Natl Acad Sci U S A. 2011. PMID: 21976485 Free PMC article.
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.
Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J, Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P. Van Goor F, et al. Among authors: frizzell ra. Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30. doi: 10.1073/pnas.0904709106. Epub 2009 Oct 21. Proc Natl Acad Sci U S A. 2009. PMID: 19846789 Free PMC article.
Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions.
Bozoky Z, Krzeminski M, Muhandiram R, Birtley JR, Al-Zahrani A, Thomas PJ, Frizzell RA, Ford RC, Forman-Kay JD. Bozoky Z, et al. Among authors: frizzell ra. Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):E4427-36. doi: 10.1073/pnas.1315104110. Epub 2013 Nov 4. Proc Natl Acad Sci U S A. 2013. PMID: 24191035 Free PMC article.
Established cell lines used in cystic fibrosis research.
Gruenert DC, Willems M, Cassiman JJ, Frizzell RA. Gruenert DC, et al. Among authors: frizzell ra. J Cyst Fibros. 2004 Aug;3 Suppl 2:191-6. doi: 10.1016/j.jcf.2004.05.040. J Cyst Fibros. 2004. PMID: 15463957 Free article. Review.
From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.
Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, Hong JS, Pollard HB, Guggino WB, Balch WE, Skach WR, Cutting GR, Frizzell RA, Sheppard DN, Cyr DM, Sorscher EJ, Brodsky JL, Lukacs GL. Veit G, et al. Among authors: frizzell ra. Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935. Mol Biol Cell. 2016. PMID: 26823392 Free PMC article.
miR-16 rescues F508del-CFTR function in native cystic fibrosis epithelial cells.
Kumar P, Bhattacharyya S, Peters KW, Glover ML, Sen A, Cox RT, Kundu S, Caohuy H, Frizzell RA, Pollard HB, Biswas R. Kumar P, et al. Among authors: frizzell ra. Gene Ther. 2015 Nov;22(11):908-16. doi: 10.1038/gt.2015.56. Epub 2015 Jul 2. Gene Ther. 2015. PMID: 26133785 Free PMC article.
CFTR Folding Consortium: methods available for studies of CFTR folding and correction.
Peters KW, Okiyoneda T, Balch WE, Braakman I, Brodsky JL, Guggino WB, Penland CM, Pollard HB, Sorscher EJ, Skach WR, Thomas PJ, Lukacs GL, Frizzell RA. Peters KW, et al. Among authors: frizzell ra. Methods Mol Biol. 2011;742:335-53. doi: 10.1007/978-1-61779-120-8_20. Methods Mol Biol. 2011. PMID: 21547742 Free PMC article.
The CFTR trafficking mutation F508del inhibits the constitutive activity of SLC26A9.
Bertrand CA, Mitra S, Mishra SK, Wang X, Zhao Y, Pilewski JM, Madden DR, Frizzell RA. Bertrand CA, et al. Among authors: frizzell ra. Am J Physiol Lung Cell Mol Physiol. 2017 Jun 1;312(6):L912-L925. doi: 10.1152/ajplung.00178.2016. Epub 2017 Mar 30. Am J Physiol Lung Cell Mol Physiol. 2017. PMID: 28360110 Free PMC article.
213 results