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Letters to the Editor
Nature Medicine  2, 608 - 609 (1996)
doi:10.1038/nm0696-608c

Reply to "Nonstop treatment of cystic fibrosis"

David M. Bedwell1, Marybeth Howard2 & Raymond A. Frizzell2

  1Department of Microbiology BBRB432 University of Alabama at Birmingham Birmingham, Alabama 35294-2170, USA

  2Department of Cell Biology and Physiology University of Pittsburgh Pittsburgh, Pennsylvania 15261, USA


REFERENCES
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  2. Maquat, L.E. When cells stop making sense: effects of nonsense codons on RNA metabolism in vertebrate cells. RNA 1, 453−465 (1995). | PubMed  | ISI | ChemPort |
  3. Dietz, H.C. et al. The skipping of constitutive exons in vivo induced by nonsense mutations. Science 259, 680−683 (1993). | PubMed  | ISI | ChemPort |
  4. Dietz, H.C. & Kendzior, R.J., Jr. Maintenance of an open reading frame as an additional level of scrutiny during splice site selection. Nature Genet. 8, 183−188 (1994). | PubMed  | ISI | ChemPort |
  5. Hamosh, A. et al. Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis. J. Clin. Invest. 88, 1880−1885 (1991). | PubMed  | ISI | ChemPort |
  6. Hamosh, A., Rosenstein, B.J. & Cutting, G.R. CFTR nonsense mutations G542X and W1282X associated with severe reduction of CFTR mRNA in nasal epithelial cells. Hum. Mol. Genet. 1, 542−544 (1992). | PubMed  | ChemPort |
  7. Hull, J.S. et al. The stop mutation R553X in the CFTR gene results in exon skipping. Genomics 19, 362−364 (1994). | Article | PubMed  | ISI | ChemPort |
  8. Dorin, J.R. et al. Phenotypic consequence of CFTR modulation in mutant mice: Implications for somatic gene therapy. Pediatr. Pulmonol. 12 (suppl.), 231 (1995).
  9. Belgrader, P., Cheng, J. & Maquat, L.E. Evidence to implicate translation by ribosomes in the mechanism by which nonsense codons reduce the nuclear level of human triosephosphate isomerase mRNA. Proc. Natl. Acad. Sci. USA 90, 482−486 (1993). | PubMed  | ChemPort |
  10. Naeger, L.K., Schoberg, R.V., Zhao, Q., Tullis, G.E. & Pintel, D.J. Nonsense mutations inhibit splicing of MVM RNA in cis when they interrupt the reading frame of either exon of the final spliced product. Genes Dev. 6, 1107−1119 (1992). | PubMed  | ISI | ChemPort |
  11. Qian, L. et al. T cell receptor-beta mRNA splicing: Regulation of unusual splicing intermediates. Mol. Cell. Biol. 13, 1686−1696 (1993). | PubMed  | ISI | ChemPort |
  12. Menon, K.P. & Neufeld, E.F. Evidence for degradation of mRNA encoding alpha-L-iduronidase in Hurler fibroblasts with premature termination alleles. Cell Mol. Biol. 40, 999−1005 (1994). | ISI | ChemPort |
  13. Chang, J.C. & Kan, Y.W. beta0 thalassemia, a nonsense mutation in man. Proc. Natl. Acad. Sci. USA 76, 2886−2889 (1979). | PubMed  | ChemPort |
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