Thibodeau PH - Search Results

1

Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation.

Pissarra LS, Farinha CM, Xu Z, Schmidt A, Thibodeau PH, Cai Z, Thomas PJ, Sheppard DN, Amaral MD. Pissarra LS, et al. Among authors: thibodeau ph. Chem Biol. 2008 Jan;15(1):62-9. doi: 10.1016/j.chembiol.2007.11.012. Chem Biol. 2008. PMID: 18215773

2

Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.

Zhang XM, Wang XT, Yue H, Leung SW, Thibodeau PH, Thomas PJ, Guggino SE. Zhang XM, et al. Among authors: thibodeau ph. J Biol Chem. 2003 Dec 19;278(51):51232-42. doi: 10.1074/jbc.M309076200. Epub 2003 Oct 7. J Biol Chem. 2003. PMID: 14532265 Free article.

3

Side chain and backbone contributions of Phe508 to CFTR folding.

Thibodeau PH, Brautigam CA, Machius M, Thomas PJ. Thibodeau PH, et al. Nat Struct Mol Biol. 2005 Jan;12(1):10-6. doi: 10.1038/nsmb881. Epub 2004 Dec 26. Nat Struct Mol Biol. 2005. PMID: 15619636 Free PMC article.

4

Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.

Xu Z, Pissarra LS, Farinha CM, Liu J, Cai Z, Thibodeau PH, Amaral MD, Sheppard DN. Xu Z, et al. Among authors: thibodeau ph. J Physiol. 2014 May 1;592(9):1931-47. doi: 10.1113/jphysiol.2014.271817. Epub 2014 Mar 3. J Physiol. 2014. PMID: 24591578 Free PMC article.

5

Cystic fibrosis: recent structural insights.

Dorwart M, Thibodeau P, Thomas P. Dorwart M, et al. J Cyst Fibros. 2004 Aug;3 Suppl 2:91-4. doi: 10.1016/j.jcf.2004.05.020. J Cyst Fibros. 2004. PMID: 15463935 Free article. Review.

6

CFTR regulatory region interacts with NBD1 predominantly via multiple transient helices.

Baker JM, Hudson RP, Kanelis V, Choy WY, Thibodeau PH, Thomas PJ, Forman-Kay JD. Baker JM, et al. Among authors: thibodeau ph. Nat Struct Mol Biol. 2007 Aug;14(8):738-45. doi: 10.1038/nsmb1278. Epub 2007 Jul 29. Nat Struct Mol Biol. 2007. PMID: 17660831 Free PMC article.

7

NMR evidence for differential phosphorylation-dependent interactions in WT and DeltaF508 CFTR.

Kanelis V, Hudson RP, Thibodeau PH, Thomas PJ, Forman-Kay JD. Kanelis V, et al. Among authors: thibodeau ph. EMBO J. 2010 Jan 6;29(1):263-77. doi: 10.1038/emboj.2009.329. Epub 2009 Nov 19. EMBO J. 2010. PMID: 19927121 Free PMC article.

8

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. 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.

9

A protein sequence that can encode native structure by disfavoring alternate conformations.

Wigley WC, Corboy MJ, Cutler TD, Thibodeau PH, Oldan J, Lee MG, Rizo J, Hunt JF, Thomas PJ. Wigley WC, et al. Among authors: thibodeau ph. Nat Struct Biol. 2002 May;9(5):381-8. doi: 10.1038/nsb784. Nat Struct Biol. 2002. PMID: 11938353

10

Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator.

Lewis HA, Buchanan SG, Burley SK, Conners K, Dickey M, Dorwart M, Fowler R, Gao X, Guggino WB, Hendrickson WA, Hunt JF, Kearins MC, Lorimer D, Maloney PC, Post KW, Rajashankar KR, Rutter ME, Sauder JM, Shriver S, Thibodeau PH, Thomas PJ, Zhang M, Zhao X, Emtage S. Lewis HA, et al. Among authors: thibodeau ph. EMBO J. 2004 Jan 28;23(2):282-93. doi: 10.1038/sj.emboj.7600040. Epub 2003 Dec 18. EMBO J. 2004. PMID: 14685259 Free PMC article.

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