Structural Studies of CFTR


Hildebrandt E, Khazanov N, Kappes JC, Dai Q, Senderowitz H, Urbatsch IL.  Specific stabilization of CFTR by phosphatidylserine.  Biochim Biophys Acta. 2016 Nov 29. pii: S0005-2736(16)30381-9.


Hildebrandt E, Ding H, Mulky A, Dai Q, Aleksandrov AA, Bajrami B, Diego PA, Wu X, Ray M, Naren AP, Riordan JR, Yao X, DeLucas LJ, Urbatsch IL, Kappes JC.  A stable human-cell system overexpressing cystic fibrosis transmembrane conductance regulator recombinant protein at the cell surface.  Mol Biotechnol. 2015 May;57(5):391-405.


Hildebrandt E, Zhang Q, Cant N, Ding H, Dai Q, Peng L, Fu Y, DeLucas LJ, Ford R, Kappes JC, Urbatsch IL.  A survey of detergents for the purification of stable, active human cystic fibrosis transmembrane conductance regulator (CFTR).  Biochim Biophys Acta. 2014 Nov;1838(11):2825-37. 


Yang Z, Wang C, Zhou Q, An J, Hildebrandt E, Aleksandrov LA, Kappes JC, DeLucas LJ, Riordan JR, Urbatsch IL, Hunt JF, Brouillette CG. Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains. Protein Sci. 2014, Jun; 23(6):769-89

Pollock N, Cant N, Rimington T, Ford RC. Purification of the cystic fibrosis transmembrane conductance regulator protein expressed in Saccharomyces cerevisiae. J Vis Exp. 2014 May 10;(87)

He L, Aleksandrov AA, An J, Cui L, Yang Z, Brouillette CG, Riordan JR. Restoration of NBD1 Thermal Stability Is Necessary and Sufficient to Correct ΔF508 CFTR Folding and Assembly. J Mol Biol. 2014 Jul 30. pii: S0022-2836

Cant N, Pollock N, Ford RC. CFTR structure and cystic fibrosis. Int J Biochem Cell Biol. 2014 Jul;52:15-25.

Hildebrandt E, Zhang Q, Cant N, Ding H, Dai Q, Peng L, Fu Y, DeLucas LJ, Ford R, Kappes JC, Urbatsch IL. A survey of detergents for the purification of stable, active human cystic fibrosis transmembrane conductance regulator (CFTR). Biochim Biophys Acta. 2014 Nov;1838(11):2825-37

Bozoky Z, Krzeminski M, Muhandiram R, Birtley JR, Al-Zahrani A, Thomas PJ, Frizzell RA, Ford RC, Forman-Kay JD. Regulatory R region of the CFTR chloride channel is a dynamic integrator of phospho-dependent intra- and intermolecular interactions. Proc Natl Acad Sci USA. 2013 Nov 19;110(47):E4427-36

J.E. Dawson, P. Farber and J.D. Forman-Kay. Allosteric Coupling between the Intracellular Coupling Helix 4 and Regulatory Sites of the First Nucleotide-binding Domain of CFTR. PLoS One, 2013 8(9):e74347

Hunt JF, Wang C, Ford RC. Cystic fibrosis transmembrane conductance regulator (ABCC7) structure. Cold Spring Harb Perspect Med. 2013 Feb 1;3(2):a009514. doi: 10.1101/cshperspect.a009514. Review. 

Hudson, R.; Chong, A.; Protasevich, I.I, Vernon, R.; Noy, E.; Bihler, H.; Li An, J.; Kalid, O.; Sela-Culang, I.; Mense, M.; Senderowitz, H.; Brouillette, C,; Forman-Kay, J.D. Conformational Changes Relevant to Channel Activity and Folding within the first Nucleotide Binding Domain of CFTR. J Biol Chem. 2012, 287, 28480-94.

Nay, E.; Senderowitz, H. Combating cystic fibrosis: in search for CF transmembrane conductance regulator (CFTR) modulators. ChemMedChem 2011, 6, 243-51

Kanelis V, Chong PA, Forman-Kay JD. NMR spectroscopy to study the dynamics and interactions of CFTR. Methods Mol Biol. 2011;741:377-403. doi: 10.1007/978-1-61779-117-8_25.

Thibodeau, P.H.; Richardson III, J.M.; Wang, W.; Millen, L.; Watson, J.; Mendoza, J.; Du, K.; Fischman, S.; Senderowitz, H.; Lukacs, G.; Kirk, K.; Thomas, P.J. The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis. JBC, 2010 285, 35825-35.

Kalid, O.; Fischman, S.; Mense, M.; Shitrit, A.; Bihler, H.; Ben-Zeev, E.; Schutz, N.; Pedemonte, N.; Thomas, P.J.; Bridges, R.J.; Wetmore, D.R.; Marantz, Y.; Senderowitz, H. Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening. J. Comput. Aided. Mol. Design, 2010, 24, 971-91. 

Protasevich I, Yang Z, Atwell S, Zhao X, Emtage S, Wetmore D, Hunt JF, Brouillette CG. Thermal unfolding studies show the disease causing F508del mutation in CFTR thermodynamically destabilizes nucleotide-binding domain 1. Protein Sci. 2010 Oct; 19(10)1917-31. PMID: 20687133. 

Wang C, Protasevich I, Yang Z, Seehausen D, Skalak T, Zhao X, Atwell S, Emtage SJ. Integrated biophysical studies implicate partial unfolding of NBD1 of CFTR in the molecular pathogenesis of F508del cystic fibrosis Protein Sci. 2010 Oct; 19(10):1932-47. PMID: 20687163.

Riordan JR. CFTR function and prospects for therapy. Annu Rev Biochem. 2008; 77:701-26. PMID 18304008 

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© 2019 CFTR 3D-Structure Consortium; Updated October 7, 2019