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Items: 18

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.

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
3.

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.

4.

Biosynthesis and degradation of CFTR.

Kopito RR.

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

5.

Novel, mechanism-based therapies for cystic fibrosis.

Rubenstein RC.

Curr Opin Pediatr. 2005 Jun;17(3):385-92. Review.

PMID:
15891431
6.

CFTR: folding, misfolding and correcting the ΔF508 conformational defect.

Lukacs GL, Verkman AS.

Trends Mol Med. 2012 Feb;18(2):81-91. doi: 10.1016/j.molmed.2011.10.003. Epub 2011 Dec 3. Review.

7.

Less is more: improving proteostasis by translation slow down.

Sherman MY, Qian SB.

Trends Biochem Sci. 2013 Dec;38(12):585-91. doi: 10.1016/j.tibs.2013.09.003. Epub 2013 Oct 11. Review.

PMID:
24126073
8.

Cystic fibrosis: a disease of altered protein folding.

Qu BH, Strickland E, Thomas PJ.

J Bioenerg Biomembr. 1997 Oct;29(5):483-90. Review.

PMID:
9511933
9.

Strategies for correcting the delta F508 CFTR protein-folding defect.

Brown CR, Hong-Brown LQ, Welch WJ.

J Bioenerg Biomembr. 1997 Oct;29(5):491-502. Review.

PMID:
9511934
10.

Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism.

Gelman MS, Kopito RR.

Methods Mol Biol. 2003;232:27-37. Review. No abstract available.

PMID:
12840537
11.

Can correcting the ΔF508-CFTR proteostasis-defect rescue CF lung disease?

Valle CW, Vij N.

Curr Mol Med. 2012 Aug;12(7):860-71. Review.

PMID:
22697346
12.

Targeting CFTR: how to treat cystic fibrosis by CFTR-repairing therapies.

Amaral MD.

Curr Drug Targets. 2011 May;12(5):683-93. Review.

PMID:
21039334
13.
14.

Proteomics uncovering possible key players in F508del-CFTR processing and trafficking.

Gomes-Alves P, Penque D.

Expert Rev Proteomics. 2010 Aug;7(4):487-94. doi: 10.1586/epr.10.37. Review.

PMID:
20653505
15.

Defective folding and rapid degradation of mutant proteins is a common disease mechanism in genetic disorders.

Gregersen N, Bross P, Jørgensen MM, Corydon TJ, Andresen BS.

J Inherit Metab Dis. 2000 Jul;23(5):441-7. Review.

PMID:
10947197
16.
17.

Type I, II, III, IV, and V cystic fibrosis transmembrane conductance regulator defects and opportunities for therapy.

Choo-Kang LR, Zeitlin PL.

Curr Opin Pulm Med. 2000 Nov;6(6):521-9. Review.

PMID:
11100963
18.

CFTR and chaperones: processing and degradation.

Amaral MD.

J Mol Neurosci. 2004;23(1-2):41-8. Review.

PMID:
15126691
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