Supplementary Materials

Supplementary Material for:

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis

Deborah M. Cholon, Nancy L. Quinney, M. Leslie Fulcher, Charles R. Esther Jr., Jhuma Das, Nikolay V. Dokholyan, Scott H. Randell, Richard C. Boucher, Martina Gentzsch*

*Corresponding author. E-mail: gentzsch@med.unc.edu

Published 23 July 2014, Sci. Transl. Med. 6, 246ra96 (2014)
DOI: 10.1126/scitranslmed.3008680

This PDF file includes:

  • Fig. S1. Chronic VX-770 treatment alters responses to amiloride and UTP in CF HBE cells.
  • Fig. S2. Chronic VX-770 treatment inhibits functional rescue of ΔF508 by VX-661.
  • Fig. S3. Chronic VX-770 treatment alters responses to amiloride and UTP in normal HBE cells.
  • Fig. S4. VX-770 and VX-809 concentrations were measured in treated HBE cells.
  • Fig. S5. G551D mutation in the NBD1 stabilizes CFTR protein.
  • Table S1. VX-770 treatment restores G551D function.
  • Table S2. Chronic VX-770 treatment alters responses to amiloride and UTP.
  • Table S3. Chronic VX-770 treatment inhibits functional rescue of ΔF508 by VX-809.
  • Table S4. Chronic VX-770 treatment inhibits functional rescue of ΔF508 by VX-661.
  • Table S5. VX-770 diminishes biochemical correction by increasing turnover of corrected ΔF508 CFTR.
  • Table S6. VX-770–induced hindrance of ΔF508 correction is dose-dependent.
  • Table S7. VX-770 at low dose (50 nM) affects ISC of VX-809–corrected CF HBE cells (ΔF508/ΔF508).
  • Table S8. VX-770 affects C/B band ratio in CF HBE (ΔF508/ΔF508).
  • Table S9. Chronic VX-770 treatment decreases the function of wild-type CFTR.
  • Table S10. Transepithelial resistance and nystatin responses were not altered in HBE cultures chronically treated with VX-770.
  • Table S11. VX-770 and VX-809 concentrations were measured in HBE cell lysates.
  • Table S12. VX-770 reduces stability of CFTR.

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