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Items: 1 to 20 of 74

1.

A novel approach to recovery of function of mutant proteins by slowing down translation.

Meriin AB, Mense M, Colbert JD, Liang F, Bihler H, Zaarur N, Rock KL, Sherman MY.

J Biol Chem. 2012 Oct 5;287(41):34264-72. doi: 10.1074/jbc.M112.397307. Epub 2012 Aug 17.

2.
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Folding of CFTR is predominantly cotranslational.

Kleizen B, van Vlijmen T, de Jonge HR, Braakman I.

Mol Cell. 2005 Oct 28;20(2):277-87.

4.

Protein synthesis. The delicate dance of translation and folding.

Puglisi JD.

Science. 2015 Apr 24;348(6233):399-400. doi: 10.1126/science.aab2157. No abstract available.

PMID:
25908811
5.
6.

Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.

Powell K, Zeitlin PL.

Adv Drug Deliv Rev. 2002 Dec 5;54(11):1395-408. Review.

PMID:
12458151
7.

Protein folding. Translational tuning optimizes nascent protein folding in cells.

Kim SJ, Yoon JS, Shishido H, Yang Z, Rooney LA, Barral JM, Skach WR.

Science. 2015 Apr 24;348(6233):444-8. doi: 10.1126/science.aaa3974.

8.

Slowing bacterial translation speed enhances eukaryotic protein folding efficiency.

Siller E, DeZwaan DC, Anderson JF, Freeman BC, Barral JM.

J Mol Biol. 2010 Mar 12;396(5):1310-8. doi: 10.1016/j.jmb.2009.12.042. Epub 2010 Jan 4.

PMID:
20043920
10.

Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.

Sloane PA, Rowe SM.

Curr Opin Pulm Med. 2010 Nov;16(6):591-7. doi: 10.1097/MCP.0b013e32833f1d00. Review.

11.

AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.

Johnson JS, Gentzsch M, Zhang L, Ribeiro CM, Kantor B, Kafri T, Pickles RJ, Samulski RJ.

PLoS Pathog. 2011 May;7(5):e1002053. doi: 10.1371/journal.ppat.1002053. Epub 2011 May 19.

12.

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.

Brown CR, Hong-Brown LQ, Biwersi J, Verkman AS, Welch WJ.

Cell Stress Chaperones. 1996 Jun;1(2):117-25.

13.

Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae.

O'Ryan L, Rimington T, Cant N, Ford RC.

J Vis Exp. 2012 Mar 10;(61). pii: 3860. doi: 10.3791/3860.

14.

Biosynthesis and degradation of CFTR.

Kopito RR.

Physiol Rev. 1999 Jan;79(1 Suppl):S167-73. Review.

15.

Rescue of F508del-CFTR by RXR motif inactivation triggers proteome modulation associated with the unfolded protein response.

Gomes-Alves P, Couto F, Pesquita C, Coelho AV, Penque D.

Biochim Biophys Acta. 2010 Apr;1804(4):856-65. doi: 10.1016/j.bbapap.2009.12.013. Epub 2010 Jan 4.

PMID:
20044041
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18.

Expression and degradation of the cystic fibrosis transmembrane conductance regulator in Saccharomyces cerevisiae.

Kiser GL, Gentzsch M, Kloser AK, Balzi E, Wolf DH, Goffeau A, Riordan JR.

Arch Biochem Biophys. 2001 Jun 15;390(2):195-205.

PMID:
11396922
19.

Investigation of folding and degradation of in vitro synthesized mutant proteins in microsomes.

Cuthbert AW, Fuller W.

Methods Mol Biol. 2003;232:265-83. No abstract available.

PMID:
12840556
20.

Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants.

Sun F, Zhang R, Gong X, Geng X, Drain PF, Frizzell RA.

J Biol Chem. 2006 Dec 1;281(48):36856-63. Epub 2006 Sep 5.

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