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Items: 1 to 20 of 48

1.

Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.

Bagdany M, Veit G, Fukuda R, Avramescu RG, Okiyoneda T, Baaklini I, Singh J, Sovak G, Xu H, Apaja PM, Sattin S, Beitel LK, Roldan A, Colombo G, Balch W, Young JC, Lukacs GL.

Nat Commun. 2017 Aug 30;8(1):398. doi: 10.1038/s41467-017-00444-4.

2.

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 25;292(34):14147-14164. doi: 10.1074/jbc.M116.772335. Epub 2017 Jun 27.

PMID:
28655774
3.

Folding correction of ABC-transporter ABCB1 by pharmacological chaperones: a mechanistic concept.

Spork M, Sohail MI, Schmid D, Ecker GF, Freissmuth M, Chiba P, Stockner T.

Pharmacol Res Perspect. 2017 May 26;5(3):e00325. doi: 10.1002/prp2.325. eCollection 2017 Jun.

4.

Codon bias and the folding dynamics of the cystic fibrosis transmembrane conductance regulator.

Bartoszewski R, Króliczewski J, Piotrowski A, Jasiecka AJ, Bartoszewska S, Vecchio-Pagan B, Fu L, Sobolewska A, Matalon S, Cutting GR, Rowe SM, Collawn JF.

Cell Mol Biol Lett. 2016 Oct 19;21:23. doi: 10.1186/s11658-016-0025-x. eCollection 2016. Review.

5.

Alteration of protein function by a silent polymorphism linked to tRNA abundance.

Kirchner S, Cai Z, Rauscher R, Kastelic N, Anding M, Czech A, Kleizen B, Ostedgaard LS, Braakman I, Sheppard DN, Ignatova Z.

PLoS Biol. 2017 May 16;15(5):e2000779. doi: 10.1371/journal.pbio.2000779. eCollection 2017 May.

6.

ABCMdb reloaded: updates on mutations in ATP binding cassette proteins.

Tordai H, Jakab K, Gyimesi G, András K, Brózik A, Sarkadi B, Hegedus T.

Database (Oxford). 2017 Jan 1;2017(1). doi: 10.1093/database/bax023.

7.

Cystic fibrosis lung environment and Pseudomonas aeruginosa infection.

Bhagirath AY, Li Y, Somayajula D, Dadashi M, Badr S, Duan K.

BMC Pulm Med. 2016 Dec 5;16(1):174. Review.

8.

Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation Product.

Haggie PM, Phuan PW, Tan JA, Xu H, Avramescu RG, Perdomo D, Zlock L, Nielson DW, Finkbeiner WE, Lukacs GL, Verkman AS.

J Biol Chem. 2017 Jan 20;292(3):771-785. doi: 10.1074/jbc.M116.764720. Epub 2016 Nov 28.

9.

CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis.

Hegyi P, Wilschanski M, Muallem S, Lukacs GL, Sahin-Tóth M, Uc A, Gray MA, Rakonczay Z Jr, Maléth J.

Rev Physiol Biochem Pharmacol. 2016;170:37-66. doi: 10.1007/112_2015_5002. Review.

10.

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.

Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935.

11.

hERG quality control and the long QT syndrome.

Foo B, Williamson B, Young JC, Lukacs G, Shrier A.

J Physiol. 2016 May 1;594(9):2469-81. doi: 10.1113/JP270531. Epub 2016 Feb 9. Review.

12.

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 22;291(4):2004-17. doi: 10.1074/jbc.M115.685628. Epub 2015 Dec 1.

13.

Hallmarks of therapeutic management of the cystic fibrosis functional landscape.

Amaral MD, Balch WE.

J Cyst Fibros. 2015 Nov;14(6):687-99. doi: 10.1016/j.jcf.2015.09.006. Epub 2015 Oct 29. Review.

14.

A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator.

Bali V, Lazrak A, Guroji P, Fu L, Matalon S, Bebok Z.

FASEB J. 2016 Jan;30(1):201-13. doi: 10.1096/fj.15-273714. Epub 2015 Sep 3.

15.

The Transmission Interfaces Contribute Asymmetrically to the Assembly and Activity of Human P-glycoprotein.

Loo TW, Clarke DM.

J Biol Chem. 2015 Jul 3;290(27):16954-63. doi: 10.1074/jbc.M115.652602. Epub 2015 May 18.

16.

Decoding mechanisms by which silent codon changes influence protein biogenesis and function.

Bali V, Bebok Z.

Int J Biochem Cell Biol. 2015 Jul;64:58-74. doi: 10.1016/j.biocel.2015.03.011. Epub 2015 Mar 26. Review.

17.

Synonymous codon usage affects the expression of wild type and F508del CFTR.

Shah K, Cheng Y, Hahn B, Bridges R, Bradbury NA, Mueller DM.

J Mol Biol. 2015 Mar 27;427(6 Pt B):1464-79. doi: 10.1016/j.jmb.2015.02.003. Epub 2015 Feb 10.

18.

Current progress of genetically engineered pig models for biomedical research.

Gün G, Kues WA.

Biores Open Access. 2014 Dec 1;3(6):255-64. doi: 10.1089/biores.2014.0039. Review.

19.

Biophysical characterisation of calumenin as a charged F508del-CFTR folding modulator.

Tripathi R, Benz N, Culleton B, Trouvé P, Férec C.

PLoS One. 2014 Aug 13;9(8):e104970. doi: 10.1371/journal.pone.0104970. eCollection 2014.

20.

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