Supplementary Materials

Supplementary Material for:

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression

Guido Veit, Radu G. Avramescu, Doranda Perdomo, Puay-Wah Phuan, Miklos Bagdany, Pirjo M. Apaja, Florence Borot, Daniel Szollosi, Yu-Sheng Wu, Walter E. Finkbeiner, Tamas Hegedus, Alan S. Verkman, Gergely L. Lukacs*

*Corresponding author. E-mail: gergely.lukacs@mcgill.ca

Published 23 July 2014, Sci. Transl. Med. 6, 246ra97 (2014)
DOI: 10.1126/scitranslmed.3008889

This PDF file includes:

  • Materials and Methods
  • Fig. S1. Comparison between 3HA- and HRP-tagged ΔF508-CFTR.
  • Fig. S2. Acute VX-770 potentiation in CFBE and the effect of extended VX-770 exposure on the PM density of rΔF508-CFTR in other epithelial cell models.
  • Fig. S3. Structure and effect of CFFT potentiator panel on the rΔF508-CFTR function and PM density.
  • Fig. S4. The effect of prolonged exposure to VX-770 on the biogenesis and stability of CFTR variants.
  • Fig. S5. The effect of VX-770 on the PM density of CFTR variants.
  • Fig. S6. Representative records of ΔF508-R1S and ΔF508-E1371S activities in artificial phospholipid bilayer.
  • Fig. S7. In silico modeling of VX-770 and P5 binding to wild-type and ΔF508-CFTR cytosolic regions.
  • Fig. S8. The effect of prolonged VX-770 exposure on the CF-causing mutants R347H-, R170G-, and P67L-CFTR.
  • Table S1. Effect of 24 hours of potentiator treatment on Isc measurements in primary CFTRWT/WT HBE.
  • Table S2. CFTR mutants used in this study.
  • References (6770)

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Other Supplementary Material for this manuscript includes the following:

  • Table S3. Data and derived P values used in composite graphs (provided as an Excel file).

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