Associated Papers
Rescue of Rare CFTR Trafficking Mutants Highlights a Structural Location-Dependent Pattern for Correction.
Zacarias S et al.
International Journal of Molecular Sciences, 2023
36834620 (PubMed )
Supplement: ijms-24-03211-s001.zip › ijms-2071953-supplementary.pdf
MPTherm-pred: Analysis and Prediction of Thermal Stability Changes upon Mutations in Transmembrane Proteins.
Kulandaisamy A et al.
Journal of Molecular Biology, 2021
Recent Strategic Advances in CFTR Drug Discovery: An Overview.
Rusnati M et al.
International Journal of Molecular Sciences, 2020
VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.
Cui G et al.
Scientific Reports, 2019
Asymmetry of movements in CFTR's two ATP sites during pore opening serves their distinct functions.
Sorum B et al.
Elife, 2017
∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis.
Pankow S et al.
Nature, 2015
ABCMdb: a database for the comparative analysis of protein mutations in ABC transporters, and a potential framework for a general application.
Gyimesi G et al.
Human Mutation, 2012
Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function.
Rabeh WM et al.
Cell, 2012
The cystic fibrosis-causing mutation deltaF508 affects multiple steps in cystic fibrosis transmembrane conductance regulator biogenesis.
Thibodeau PH et al.
The Journal of Biological Chemistry, 2010
Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry.
Lewis HA et al.
Journal of Molecular Biology, 2010
Building an understanding of cystic fibrosis on the foundation of ABC transporter structures.
Mendoza JL et al.
Journal of Bioenergetics and Biomembranes, 2007
Side chain and backbone contributions of Phe508 to CFTR folding.
Thibodeau PH et al.
Nature Structural & Molecular Biology, 2005
[Report of a patient with Lesch-Nyhan syndrome caused by total deficiency of HGPRT and with normal activity in female family members].
Ferrández A et al.
Anales Espanoles De Pediatria, 1982
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