Focus
The focus of the CFTR Folding Consortium is to:
- Understand the process of CFTR folding and trafficking
- Develop and distribute reagents (antibodies and proteins) and methods to facilitate a practical understanding of why disease-associated CFTR mutant misfolds and how to correct these defects
Consortium Membership
Resources
Methods and reagents are available to researchers focused on understanding why disease-associated CFTR mutations misfold and how to correct the defects.
Investigators who utilize available antibody and protein resources are required to provide feedback on their utility and are asked to make available any new, improved reagents or methods they developed from the resources.
Suggested Reading
- Du K, Lukacs GL. Cooperative assembly and misfolding of CFTR domains in vivo. Mol Biol Cell. 2009 Apr;20(7):1903-15. Epub 2009 Jan 28.PMID: 19176754
- Kleizen B, van Vlijmen T, de Jonge HR, Braakman I. Folding of CFTR is predominantly cotranslational. Mol Cell. 2005 Oct 28;20(2):277-87.PMID: 16246729
- Nakatsukasa K, Huyer G, Michaelis S, Brodsky JL. Dissecting the ER-associated degradation of a misfolded polytopic membrane protein. Cell. 2008 Jan 11;132(1):101-12.PMID: 18191224
- Caohuy H, Jozwik C, Pollard HB. Rescue of DeltaF508-CFTR by the SGK1/Nedd4-2 signaling pathway. J Biol Chem. 2009 Sep 11;284(37):25241-53. Epub 2009 Jul 17.PMID: 19617352
- Cebotaru L, Vij N, Ciobanu I, Wright J, Flotte T, Guggino WB. Cystic fibrosis transmembrane regulator missing the first four transmembrane segments increases wild type and DeltaF508 processing. J Biol Chem. 2008 Aug 8;283(32):21926-33. Epub 2008 May 28.PMID: 18508776
- Mendoza JL, Schmidt A, Li Q, Nuvaga E, Barrett T, Bridges RJ, Feranchak AP, Brautigam CA, Thomas PJ. Requirements for efficient correction of ΔF508 CFTR revealed by analyses of evolved sequences. Cell. 2012 Jan 20;148(1-2):164-74.PMID:22265409
- Peters KW, Okiyoneda T, Balch WE, Braakman I, Brodsky JL, Guggino WB, Penland CM, Pollard HB, Sorscher EJ, Skach WR, Thomas PJ, Lukacs GL, Frizzell RA. CFTR Folding Consortium: methods available for studies of CFTR folding and correction. Methods Mol Biol. 2011;742:335-53.PMID:21547742
- Rabeh WM, Bossard F, Xu H, Okiyoneda T, Bagdany M, Mulvihill CM, Du K, di Bernardo S, Liu Y, Konermann L, Roldan A, Lukacs GL. Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function. Cell. 2012 Jan 20;148(1-2):150-63.PMID:22265408