296 related articles for article (PubMed ID: 20687163)
1. 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. PubMed ID: 20687163
[TBL] [Abstract][Full Text] [Related]
2. 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. PubMed ID: 20687133
[TBL] [Abstract][Full Text] [Related]
3. 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; 280(2):1346-53. PubMed ID: 15528182
[TBL] [Abstract][Full Text] [Related]
4. Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation.
Gong X; Ahner A; Roldan A; Lukacs GL; Thibodeau PH; Frizzell RA
J Biol Chem; 2016 Jan; 291(4):2004-2017. PubMed ID: 26627832
[TBL] [Abstract][Full Text] [Related]
5. 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. PubMed ID: 18215773
[TBL] [Abstract][Full Text] [Related]
6. 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. PubMed ID: 19878303
[TBL] [Abstract][Full Text] [Related]
7. Decoding F508del misfolding in cystic fibrosis.
Wang XR; Li C
Biomolecules; 2014 May; 4(2):498-509. PubMed ID: 24970227
[TBL] [Abstract][Full Text] [Related]
8. 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; 286(28):24714-25. PubMed ID: 21602569
[TBL] [Abstract][Full Text] [Related]
9. Deletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.
Chong PA; Farber PJ; Vernon RM; Hudson RP; Mittermaier AK; Forman-Kay JD
J Biol Chem; 2015 Sep; 290(38):22862-78. PubMed ID: 26149808
[TBL] [Abstract][Full Text] [Related]
10. 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; 427(1):106-20. PubMed ID: 25083918
[TBL] [Abstract][Full Text] [Related]
11. 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. PubMed ID: 23924900
[TBL] [Abstract][Full Text] [Related]
12. Can two wrongs make a right? F508del-CFTR ion channel rescue by second-site mutations in its transmembrane domains.
Prins S; Corradi V; Sheppard DN; Tieleman DP; Vergani P
J Biol Chem; 2022 Mar; 298(3):101615. PubMed ID: 35065958
[TBL] [Abstract][Full Text] [Related]
13. REMD Simulations Reveal the Dynamic Profile and Mechanism of Action of Deleterious, Rescuing, and Stabilizing Perturbations to NBD1 from CFTR.
Zhenin M; Noy E; Senderowitz H
J Chem Inf Model; 2015 Nov; 55(11):2349-64. PubMed ID: 26418372
[TBL] [Abstract][Full Text] [Related]
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; 103(47):17891-6. PubMed ID: 17098864
[TBL] [Abstract][Full Text] [Related]
15. Thermal unfolding simulations of NBD1 domain variants reveal structural motifs associated with the impaired folding of F508del-CFTR.
Estácio SG; Martiniano HF; Faísca PF
Mol Biosyst; 2016 Aug; 12(9):2834-48. PubMed ID: 27354240
[TBL] [Abstract][Full Text] [Related]
16. Domain-interface dynamics of CFTR revealed by stabilizing nanobodies.
Sigoillot M; Overtus M; Grodecka M; Scholl D; Garcia-Pino A; Laeremans T; He L; Pardon E; Hildebrandt E; Urbatsch I; Steyaert J; Riordan JR; Govaerts C
Nat Commun; 2019 Jun; 10(1):2636. PubMed ID: 31201318
[TBL] [Abstract][Full Text] [Related]
17. Binding screen for cystic fibrosis transmembrane conductance regulator correctors finds new chemical matter and yields insights into cystic fibrosis therapeutic strategy.
Hall JD; Wang H; Byrnes LJ; Shanker S; Wang K; Efremov IV; Chong PA; Forman-Kay JD; Aulabaugh AE
Protein Sci; 2016 Feb; 25(2):360-73. PubMed ID: 26444971
[TBL] [Abstract][Full Text] [Related]
18. Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry.
Lewis HA; Wang C; Zhao X; Hamuro Y; Conners K; Kearins MC; Lu F; Sauder JM; Molnar KS; Coales SJ; Maloney PC; Guggino WB; Wetmore DR; Weber PC; Hunt JF
J Mol Biol; 2010 Feb; 396(2):406-30. PubMed ID: 19944699
[TBL] [Abstract][Full Text] [Related]
19. 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; 18(2):231-42. PubMed ID: 21338920
[TBL] [Abstract][Full Text] [Related]
20. Stabilization of a nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator yields insight into disease-causing mutations.
Vernon RM; Chong PA; Lin H; Yang Z; Zhou Q; Aleksandrov AA; Dawson JE; Riordan JR; Brouillette CG; Thibodeau PH; Forman-Kay JD
J Biol Chem; 2017 Aug; 292(34):14147-14164. PubMed ID: 28655774
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]