Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin
J. Clin. Invest. Liat Linde, et al. 117:683 doi:10.1172/JCI28523 [
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Figure 2Effect of NMD inhibition on the level of endogenous
CFTR transcripts.
(
A) Effect of indirect NMD inhibition by CHX on the level of
CFTR transcripts. The level was measured by real-time PCR and normalized to that of
RPS9 and the ratio between these normalized levels following treatment was calculated and compared to the ratio in untreated cells. (
B) Western blot analysis in CFP15a (upper panels) and CFP15b (lower panel) cells following sequence-specific downregulation of UPF1 and UPF2. (
C and
D) Effect of direct NMD inhibition by siRNA directed against
UPF1 (
C) and
UPF2 (
D) on the level of
CFTR transcript. The level was measured and normalized as described in
A. The increase in the level of
CFTR transcripts is shown as mean ± SEM.
