Myotonic dystrophy is caused by expanded CTG repeats, and the expression of these repeats as RNA leads to the sequestration of the splicing factor muscleblind-like (MBNL1) to the CUG RNA. Two mouse models for myotonic dystrophy—mice expressing expanded CUG repeats and mice lacking functional MBNL1—now reveal ∼100 new mis-splicing events and a new class of aberrantly regulated mRNAs.
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References
Cooper, T.A., Wan, L. & Dreyfuss, G. Cell 136, 777–793 (2009).
O'Rourke, J.R. & Swanson, M.S. J. Biol. Chem. 284, 7419–7423 (2009).
Mankodi, A. et al. Science 289, 1769–1772 (2000).
Kanadia, R.N. et al. Science 302, 1978–1980 (2003).
Du, H. et al. Nat. Struct. Mol. Biol. 17, 187–193 (2010).
Goers, E.S., Purcell, J., Voelker, R.B., Gates, D.P. & Berglund, J.A. Nucleic Acids Res. doi:10.1093/nar/gkp1209 (in the press).
Licatalosi, D.D. et al. Nature 456, 464–469 (2008).
Yeo, G.W. et al. Nat. Struct. Mol. Biol. 16, 130–137 (2009).
Kalsotra, A. et al. Proc. Natl. Acad. Sci. USA 105, 20333–20338 (2008).
Adereth, Y., Dammai, V., Kose, N., Li, R. & Hsu, T. Nat. Cell Biol. 7, 1240–1247 (2005).
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Voelker, R., Berglund, J. Two ways to misregulate mRNAs in myotonic dystrophy. Nat Struct Mol Biol 17, 141–142 (2010). https://doi.org/10.1038/nsmb0210-141
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DOI: https://doi.org/10.1038/nsmb0210-141