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Items: 1 to 20 of 263

1.

Misfolding of the cystic fibrosis transmembrane conductance regulator and disease.

Cheung JC, Deber CM.

Biochemistry. 2008 Feb 12;47(6):1465-73. doi: 10.1021/bi702209s. Epub 2008 Jan 15. Review.

PMID:
18193900
2.

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.

3.
4.

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.

6.
8.

Destabilization of the transmembrane domain induces misfolding in a phenotypic mutant of cystic fibrosis transmembrane conductance regulator.

Choi MY, Partridge AW, Daniels C, Du K, Lukacs GL, Deber CM.

J Biol Chem. 2005 Feb 11;280(6):4968-74. Epub 2004 Nov 10.

9.

The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.

Du K, Sharma M, Lukacs GL.

Nat Struct Mol Biol. 2005 Jan;12(1):17-25. Epub 2004 Dec 26.

PMID:
15619635
10.

Building an understanding of cystic fibrosis on the foundation of ABC transporter structures.

Mendoza JL, Thomas PJ.

J Bioenerg Biomembr. 2007 Dec;39(5-6):499-505.

PMID:
18080175
11.

Insight into cystic fibrosis by structural modelling of CFTR first nucleotide binding fold (NBF1).

Annereau JP, Stoven V, Bontems F, Barthe J, Lenoir G, Blanquet S, Lallemand JY.

C R Acad Sci III. 1997 Feb;320(2):113-21.

PMID:
9181119
12.

Nucleotide binding domains of human CFTR: a structural classification of critical residues and disease-causing mutations.

Eudes R, Lehn P, Férec C, Mornon JP, Callebaut I.

Cell Mol Life Sci. 2005 Sep;62(18):2112-23.

PMID:
16132229
13.

A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator.

Annereau JP, Wulbrand U, Vankeerberghen A, Cuppens H, Bontems F, Tümmler B, Cassiman JJ, Stoven V.

FEBS Lett. 1997 May 5;407(3):303-8.

14.

A monoclonal antibody prevents aggregation of the NBD1 domain of the cystic fibrosis transmembrane conductance regulator.

Lovato V, Roesli C, Ahlskog J, Scheuermann J, Neri D.

Protein Eng Des Sel. 2007 Dec;20(12):607-14. Epub 2007 Nov 30.

PMID:
18055505
16.

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
17.

Domain-domain associations in cystic fibrosis transmembrane conductance regulator.

Wang W, He Z, O'Shaughnessy TJ, Rux J, Reenstra WW.

Am J Physiol Cell Physiol. 2002 May;282(5):C1170-80.

18.

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.

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