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References
Howard, M., Frizzell, R.A. & Bedwell, D.M. Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations. Nature Med. 2, 467–469 (1996).
Maquat, L.E. When cells stop making sense: effects of nonsense codons on RNA metabolism in vertebrate cells. RNA 1, 453–465 (1995).
Dietz, H.C. et al. The skipping of constitutive exons in vivo induced by nonsense mutations. Science 259, 680–683 (1993).
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).
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).
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).
Hull, J.S. et al. The stop mutation R553X in the CFTR gene results in exon skipping. Genomics 19, 362–364 (1994).
Dorin, J.R. et al. Phenotypic consequence of CFTR modulation in mutant mice: Implications for somatic gene therapy. Pediatr. Pulmonol. 12 (suppl.), 231 (1995).
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).
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).
Qian, L. et al. T cell receptor-β mRNA splicing: Regulation of unusual splicing intermediates. Mol. Cell. Biol. 13, 1686–1696 (1993).
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).
Chang, J.C. & Kan, Y.W. β0 thalassemia, a nonsense mutation in man. Proc. Natl. Acad. Sci. USA 76, 2886–2889 (1979).
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Bedwell, D., Howard, M. & Frizzell, R. Reply to “Nonstop treatment of cystic fibrosis”. Nat Med 2, 608–609 (1996). https://doi.org/10.1038/nm0696-608c
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DOI: https://doi.org/10.1038/nm0696-608c
