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Address correspondence to: Garry Cutting, The Johns Hopkins Medical Institutions, 733 North Broadway, BRB 559, Baltimore, Maryland 21205, USA. Phone: 410.614.0211; Email: gcutting@jhmi.edu
STH designed studies for CFBE cells, generated and analyzed data for CFBE cells, and wrote the manuscript.
AR designed studies for FRT cells, generated and analyzed data for FRT cells, and reviewed the manuscript.
MJP, EFD, AFM, TAE, ATJ, and ZL contributed to collection of data for CFBE cells. ZC performed all single-channel experiments.
KSR provided information on missense variants found in the CF population.
JH and EJS designed studies for FRT cells and reviewed the manuscript.
DNS designed studies for single-channel experiments and reviewed the manuscript.
GRC was responsible for overall study design, development of the CFBE cell expression system, critical review of all results, and writing and editing the manuscript.
We thank the CFTR2 project for variant data, R.J. Bridges and CFFT for panels of small molecule CFTR potentiators and correctors, J.R. Riordan and CFFT for anti-CFTR antibodies, P. Thomas and L. Millen for N1303K cDNA construct used for single-channel studies, C.A. Cotton for identification of the L145H variant, M.J. Welsh for isogenic FRT cell lines, and F. Van Goor and Vertex Pharmaceuticals for access to ivacaftor response data generated using FRT cells.
This work was supported by the NIH (R01DK44003), CF Foundation (Cuttin13A1, Cuttin15XX0, and Cuttin16IO to GRC; SORSCH13XX0 and SORSCH14XX0 to EJS), and CFFT (SHEPPA14XX0) to DNS.
© 2018, American Society for Clinical Investigation