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Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis.
Hutt DM, Herman D, Rodrigues AP, Noel S, Pilewski JM, Matteson J, Hoch B, Kellner W, Kelly JW, Schmidt A, Thomas PJ, Matsumura Y, Skach WR, Gentzsch M, Riordan JR, Sorscher EJ, Okiyoneda T, Yates JR 3rd, Lukacs GL, Frizzell RA, Manning G, Gottesfeld JM, Balch WE. Hutt DM, et al. Among authors: thomas pj. Nat Chem Biol. 2010 Jan;6(1):25-33. doi: 10.1038/nchembio.275. Epub 2009 Dec 6. Nat Chem Biol. 2010. PMID: 19966789 Free PMC article.
The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.
Thibodeau PH, Richardson JM 3rd, Wang W, Millen L, Watson J, Mendoza JL, Du K, Fischman S, Senderowitz H, Lukacs GL, Kirk K, Thomas PJ. Thibodeau PH, et al. Among authors: thomas pj. J Biol Chem. 2010 Nov 12;285(46):35825-35. doi: 10.1074/jbc.M110.131623. Epub 2010 Jul 28. J Biol Chem. 2010. PMID: 20667826 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: thomas pj. 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.
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: thomas pj. 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.
512 results