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Items: 1 to 20 of 55

1.

Protein kinase CK2, cystic fibrosis transmembrane conductance regulator, and the deltaF508 mutation: F508 deletion disrupts a kinase-binding site.

Treharne KJ, Crawford RM, Xu Z, Chen JH, Best OG, Schulte EA, Gruenert DC, Wilson SM, Sheppard DN, Kunzelmann K, Mehta A.

J Biol Chem. 2008 Sep 5;283(36):25103. No abstract available.

2.
3.

The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator.

Chen EY, Bartlett MC, Loo TW, Clarke DM.

J Biol Chem. 2004 Sep 17;279(38):39620-7. Epub 2004 Jul 21.

4.
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.

7.
8.

A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.

Clarke LL, Gawenis LR, Hwang TC, Walker NM, Gruis DB, Price EM.

Am J Physiol Cell Physiol. 2004 Jul;287(1):C192-9. Epub 2004 Mar 17.

PMID:
15028554
9.

Cystic fibrosis with three mutations in the cystic fibrosis transmembrane conductance regulator gene.

Dörk T, Wulbrand U, Richter T, Neumann T, Wolfes H, Wulf B, Maass G, Tümmler B.

Hum Genet. 1991 Aug;87(4):441-6.

PMID:
1715308
10.

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 19;396(2):406-30. doi: 10.1016/j.jmb.2009.11.051. Epub 2009 Nov 26.

PMID:
19944699
11.
12.
13.

Frequency of the deltaF508 mutation in 108 cystic fibrosis patients in Sao Paulo: comparison with reported Brazilian data.

Okay TS, Oliveira WP, Raiz-Júnior R, Rodrigues JC, Del Negro GM.

Clinics (Sao Paulo). 2005 Apr;60(2):131-4. Epub 2005 Apr 26.

15.

A novel natural product compound enhances cAMP-regulated chloride conductance of cells expressing CFTR[delta]F508.

deCarvalho AC, Ndi CP, Tsopmo A, Tane P, Ayafor J, Connolly JD, Teem JL.

Mol Med. 2002 Feb;8(2):75-87.

16.
17.

Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.

Okiyoneda T, Wada I, Jono H, Shuto T, Yoshitake K, Nakano N, Nagayama S, Harada K, Isohama Y, Miyata T, Kai H.

FEBS Lett. 2002 Aug 28;526(1-3):87-92.

18.

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.

Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ.

Nature. 1992 Aug 27;358(6389):761-4.

PMID:
1380673
19.

Proinflammatory effect of sodium 4-phenylbutyrate in deltaF508-cystic fibrosis transmembrane conductance regulator lung epithelial cells: involvement of extracellular signal-regulated protein kinase 1/2 and c-Jun-NH2-terminal kinase signaling.

Roque T, Boncoeur E, Saint-Criq V, Bonvin E, Clement A, Tabary O, Jacquot J.

J Pharmacol Exp Ther. 2008 Sep;326(3):949-56. doi: 10.1124/jpet.107.135186. Epub 2008 Jun 23.

20.

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.

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