Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome.

@article{Gong2007NonsenseMI,
  title={Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome.},
  author={Qiuming Gong and Li Zhang and G. Michael Vincent and Benjamin D. Horne and Zhengfeng Zhou},
  journal={Circulation},
  year={2007},
  volume={116 1},
  pages={17-24}
}
BACKGROUND Long-QT syndrome type 2 (LQT2) is caused by mutations in the human ether-a-go-go-related gene (hERG). More than 30% of the LQT2 mutations result in premature termination codons. Degradation of premature termination codon-containing mRNA transcripts by nonsense-mediated mRNA decay is increasingly recognized as a mechanism for reducing mRNA levels in a variety of human diseases. However, the role of nonsense-mediated mRNA decay in LQT2 mutations has not been explored. METHODS AND… CONTINUE READING

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