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Results: 1 to 20 of 185

Similar articles for PubMed (Select 20687133)

1.

Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1.

Protasevich I, Yang Z, Wang C, Atwell S, Zhao X, Emtage S, Wetmore D, Hunt JF, Brouillette CG.

Protein Sci. 2010 Oct;19(10):1917-31. doi: 10.1002/pro.479.

2.

Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis.

Wang C, Protasevich I, Yang Z, Seehausen D, Skalak T, Zhao X, Atwell S, Spencer Emtage J, Wetmore DR, Brouillette CG, Hunt JF.

Protein Sci. 2010 Oct;19(10):1932-47. doi: 10.1002/pro.480.

3.

Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone.

Scott-Ward TS, Amaral MD.

FEBS J. 2009 Dec;276(23):7097-109. doi: 10.1111/j.1742-4658.2009.07421.x. Epub 2009 Oct 29.

PMID:
19878303
4.

Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR.

Sampson HM, Robert R, Liao J, Matthes E, Carlile GW, Hanrahan JW, Thomas DY.

Chem Biol. 2011 Feb 25;18(2):231-42. doi: 10.1016/j.chembiol.2010.11.016.

5.

Impact of the deltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure.

Lewis HA, Zhao X, Wang C, Sauder JM, Rooney I, Noland BW, Lorimer D, Kearins MC, Conners K, Condon B, Maloney PC, Guggino WB, Hunt JF, Emtage S.

J Biol Chem. 2005 Jan 14;280(2):1346-53. Epub 2004 Nov 3.

6.

A chemical corrector modifies the channel function of F508del-CFTR.

Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE.

Mol Pharmacol. 2010 Sep;78(3):411-8. doi: 10.1124/mol.110.065862. Epub 2010 May 25.

7.

VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.

Ren HY, Grove DE, De La Rosa O, Houck SA, Sopha P, Van Goor F, Hoffman BJ, Cyr DM.

Mol Biol Cell. 2013 Oct;24(19):3016-24. doi: 10.1091/mbc.E13-05-0240. Epub 2013 Aug 7.

9.

Decoding F508del misfolding in cystic fibrosis.

Wang XR, Li C.

Biomolecules. 2014 May 6;4(2):498-509. doi: 10.3390/biom4020498. Review.

10.

The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.

Du K, Sharma M, Lukacs GL.

Nat Struct Mol Biol. 2005 Jan;12(1):17-25. Epub 2004 Dec 26.

PMID:
15619635
11.

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.

Chem Biol. 2008 Jan;15(1):62-9. doi: 10.1016/j.chembiol.2007.11.012.

12.
13.

Side chain and backbone contributions of Phe508 to CFTR folding.

Thibodeau PH, Brautigam CA, Machius M, Thomas PJ.

Nat Struct Mol Biol. 2005 Jan;12(1):10-6. Epub 2004 Dec 26.

14.

Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.

Roxo-Rosa M, Xu Z, Schmidt A, Neto M, Cai Z, Soares CM, Sheppard DN, Amaral MD.

Proc Natl Acad Sci U S A. 2006 Nov 21;103(47):17891-6. Epub 2006 Nov 10.

15.

L-phenylalanine binding and domain organization in human phenylalanine hydroxylase: a differential scanning calorimetry study.

Thórólfsson M, Ibarra-Molero B, Fojan P, Petersen SB, Sanchez-Ruiz JM, Martínez A.

Biochemistry. 2002 Jun 18;41(24):7573-85.

PMID:
12056888
16.

Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.

Yu W, Kim Chiaw P, Bear CE.

J Biol Chem. 2011 Jul 15;286(28):24714-25. doi: 10.1074/jbc.M111.239699. Epub 2011 May 21.

17.

Restoration of NBD1 thermal stability is necessary and sufficient to correct ∆F508 CFTR folding and assembly.

He L, Aleksandrov AA, An J, Cui L, Yang Z, Brouillette CG, Riordan JR.

J Mol Biol. 2015 Jan 16;427(1):106-20. doi: 10.1016/j.jmb.2014.07.026. Epub 2014 Jul 30.

PMID:
25083918
18.

Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction.

Farinha CM, King-Underwood J, Sousa M, Correia AR, Henriques BJ, Roxo-Rosa M, Da Paula AC, Williams J, Hirst S, Gomes CM, Amaral MD.

Chem Biol. 2013 Jul 25;20(7):943-55. doi: 10.1016/j.chembiol.2013.06.004.

19.
20.

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.

J Biol Chem. 2010 Nov 12;285(46):35825-35. doi: 10.1074/jbc.M110.131623. Epub 2010 Jul 28.

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