Title: Differential thermostability and response to cystic fibrosis transmembrane conductance regulator (CFTR) potentiators of human and mouse F508del-CFTR

Samuel J. Bose1 *, Marcel J. Bijvelds2 *, Yiting Wang1 *, Jia Liu1, Zhiwei Cai1, 
Alice G.M. Bot2, Hugo R. de Jonge2 † and David N. Sheppard1 †

1 School of Physiology, Pharmacology and Neuroscience, University of Bristol, Bristol, UK,
and
2 Department of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands

Running Title:	Ivacaftor potentiates human, but not mouse, F508del-CFTR

Author contributions: *SJB, MJB & YW are co-first author.  †HRdJ & DNS are co-last author

Address Correspondence to: 	H.R. de Jonge, Ph.D.
				Erasmus University Medical Center
				Department of Gastroenterology and Hepatology
				3000 CA Rotterdam
				The Netherlands
				Tel:  +31 10 703 1492
				E-mail:  h.dejonge@erasmusmc.nl
and
				D.N. Sheppard, Ph.D.
				University of Bristol
				School of Physiology, Pharmacology and Neuroscience
				Biomedical Sciences Building
				University Walk
				Bristol BS8 1TD
				United Kingdom
				Tel:  +44 117 331 2290
				E-mail:  D.N.Sheppard@bristol.ac.uk
 
Note: Data files are arranged in folders corresponding to each figure in the manuscript.

Note: No data is shown for Figure 5 as this figure consists of example traces only.

Data for Figures 8, 9, 10 and 11 were supplied by MJ Bijvelds, AGM Bot and HR de Jonge, Erasmus University Medical Centre.
Data for all other figures was supplied by SJ Bose, Y Wang, J Liu, Z Cai and DN Sheppard, University of Bristol.
All data is published with permission from the authors, January 2019.

File inventory

Folder PATH listing for volume CFTR_CF_mutants_and_drugs
Volume serial number is 9DB1-CCA0
Z:.
|   readme.txt
|   
\---Source data
    +---Figure 12
    |       Source data for Figure 12.csv
    |       
    +---Figure 3
    |       Source data for Figure 3.csv
    |       
    +---Figure 9
    |       Source data for Figure 9.csv
    |       
    +---Figure 11
    |       Source data for Figure 11.csv
    |       
    +---Figure 2
    |       Source data for Figure 2.csv
    |       
    +---Figure 6
    |       Source data for Figure 6.csv
    |       
    +---Figure 10
    |       Source data for Figure 10.csv
    |       
    +---Figure 4
    |       Source Data for Figure 4.csv
    |       
    +---Figure 8
    |       Source data for Figure 8.csv
    |       
    +---Figure 1
    |       Source data for Figure 1.csv
    |       
    \---Figure 7
            Source data for Figure 7.csv