Figure 4. CFTR-W1282X mRNA abundance is increased in the IB3-1 bronchial epithelial cell line following G418 treatment.

Figure 4

CFTR-W1282X mRNA abundance is increased in the IB3-1 bronchial epithelial cell line following G418 treatment. IB3-1 cells carry the W1282X premature stop mutation on one CFTR allele and the ΔF508 mutation on the other CFTR allele. The ΔF508 allele harbors a 3-nucleotide deletion of codon 508, so translation proceeds to the normal translation termination codon and a normal level of mRNA is produced.160 In contrast, the W1282X allele contains a nonsense mutation at codon 1282 that results in NMD.161,162 For this experiment, IB3-1 cells were grown in the presence or absence of G418, and the abundance of mRNA from each CFTR allele was determined by allele-specific oligonucleotide hybridization. The level of CFTR mRNA from the ΔF508 allele was defined as 100%, and the level of mRNA from the W1282X allele was normalized to the ΔF508 level. Each value represents the mean ± standard deviation of four independent measurements.

From: Therapies of Nonsense-Associated Diseases

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