Arrest of CFTRΔF508 folding

被引：21

作者：

Cyr, DM ^{[1
]}

机构：

[1] Univ N Carolina, Dept Cell & Dev Biol, Chapel Hill, NC 27599 USA

[2] Univ N Carolina, N Carolina Cyst Fibrosis Res Ctr, Chapel Hill, NC 27599 USA

来源：

NATURE STRUCTURAL & MOLECULAR BIOLOGY | 2005年 / 12卷 / 01期

关键词：

D O I：

10.1038/nsmb0105-2

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

The deletion of residue 508 in CFTR is the most common cystic fibrosis-causing mutation. Recent studies indicate that the main chain and side chain of this residue contribute to the proper folding of CFTR at different stages.

引用

页码：2 / 3

页数：2

共 50 条

[1] Arrest of CFTRΔF508 folding
Douglas M Cyr
Nature Structural & Molecular Biology, 2005, 12 : 2 - 3
[2] MECHANISMS FOR RESCUE OF CORRECTABLE FOLDING DEFECTS IN CFTRΔF508
Grove, D.
Rosser, M. F.
Ren, H.
Cyr, D. M.
PEDIATRIC PULMONOLOGY, 2009, : 229 - 229
[3] Mechanisms for Rescue of Correctable Folding Defects in CFTRΔF508
Grove, Diane E.
Rosser, Meredith F. N.
Ren, Hong Yu
Naren, Anjaparavanda P.
Cyr, Douglas M.
MOLECULAR BIOLOGY OF THE CELL, 2009, 20 (18) : 4059 - 4069
[4] CFTR: folding, misfolding and correcting the ΔF508 conformational defect
Lukacs, Gergely L.
Verkman, A. S.
TRENDS IN MOLECULAR MEDICINE, 2012, 18 (02) : 81 - 91
[5] Strategies for correcting the ΔF508 CFTR protein-folding defect
Brown, CR
Hong-Brown, LQ
Welch, WJ
JOURNAL OF BIOENERGETICS AND BIOMEMBRANES, 1997, 29 (05) : 491 - 502
[6] Therapeutic approaches to repair defects in ΔF508 CFTR folding and cellular targeting
Powell, K
Zeitlin, PL
ADVANCED DRUG DELIVERY REVIEWS, 2002, 54 (11) : 1395 - 1408
[7] CFTRΔF508/ΔF508 PIGS DEVELOP A PHENOTYPE LIKE THAT OF CFTR-/- PIGS AND HUMANS WITH CF
Ostedgaard, L. S.
Chen, J.
Meyerholz, D. K.
Karp, P.
Rokhlina, T.
Pezzulo, A. A.
Ernst, S.
Ludwig, P.
Launspach, J.
Samuel, M.
Prather, R.
McCray, P.
Zabner, J.
Stoltz, D.
Welsh, M.
PEDIATRIC PULMONOLOGY, 2010, : 294 - 295
[8] ENDOGENOUS PORCINE ΔF508/ΔF508 CFTR MEDIATES TRANSPORT IN THYROID EPITHELIUM
Li, H.
Ganta, S.
Fong, P.
PEDIATRIC PULMONOLOGY, 2010, : 293 - 293
[9] Evolution of the ΔF508 CFTR mutation:: Response
Guggino, SE
TRENDS IN MICROBIOLOGY, 1999, 7 (02) : 58 - 58
[10] Correcting the trafficking defect of ΔF508 CFTR
Thomas, D.
BIOCHEMISTRY AND CELL BIOLOGY-BIOCHIMIE ET BIOLOGIE CELLULAIRE, 2010, 88 (02): : 409 - 409

← 1 2 3 4 5 →