Supplementary Materials

The PDF file includes:

  • Materials and Methods
  • Fig. S1. Generation of G551D-CFTR ferrets using AAV-directed mutagenesis and somatic cell nuclear transfer.
  • Fig. S2. Forskolin-induced swelling of intestinal organoids and Isc of intestinal epithelia and PDE.
  • Fig. S3. Expression of CFTR mRNA in the fetal intestine and pancreas.
  • Fig. S4. Lung histology of all lobes from a CFTRG551D/KO ferret removed from VX-770 and a CFTRG551D/G551D ferret maintained on VX-770.
  • Fig. S5. Withdrawal of VX-770 from adult CFTRG551D/G551D ferrets leads to PI and variable degrees of glucose intolerance.
  • Fig. S6. Lung histology of CFTRG551D/KO and CFTRG551D/G551D ferrets maintained on VX-770 for life.
  • Legend for table S1
  • Legends for data files S1 and S2
  • References (3035)

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Other Supplementary Material for this manuscript includes the following:

  • Table S1. Primary data (provided as a separate Excel file).
  • Data file S1. Proteomics data (provided as a separate Excel file).
  • Data file S2 (.docx format). RNA tissue quantification and smFISH probe maps.