Mechanism-based corrector combination restores ΔF508-CFTR folding and function-Reference-Cited by-同舟云学术

Mechanism-based corrector combination restores ΔF508-CFTR folding and function

Published:2013-05-12 Issue:7 Volume:9 Page:444-454
ISSN:1552-4450
Container-title:Nature Chemical Biology
language:en
Short-container-title:Nat Chem Biol

Author:

Okiyoneda Tsukasa,Veit Guido,Dekkers Johanna F,Bagdany Miklos,Soya Naoto,Xu Haijin,Roldan Ariel,Verkman Alan S,Kurth Mark,Simon Agnes,Hegedus Tamas,Beekman Jeffrey M,Lukacs Gergely L

Publisher

Springer Science and Business Media LLC

Subject

Cell Biology,Molecular Biology

Link

https://www.nature.com/articles/nchembio.1253.pdf

Reference60 articles.

1. Riordan, J.R. CFTR function and prospects for therapy. Annu. Rev. Biochem. 77, 701–726 (2008).

2. He, L. et al. Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating. J. Biol. Chem. 283, 26383–26390 (2008).

3. Serohijos, A.W. et al. Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function. Proc. Natl. Acad. Sci. USA 105, 3256–3261 (2008).

4. Kim, S.J. & Skach, W.R. Mechanisms of CFTR folding at the endoplasmic reticulum. Front. Pharmacol. 3, 201 (2012).

5. Du, K., Sharma, M. & Lukacs, G.L. The ΔF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR. Nat. Struct. Mol. Biol. 12, 17–25 (2005).

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