MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1.

@article{Haro2006MBNL1AC,
  title={MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1.},
  author={Mar{\'i}a de Haro and Ismael Al-Ramahi and Beatrice de Gouyon and Lubna Ukani and Alberto Mar{\'i}a Rosa and Nuno Andr{\'e} Faustino and Tetsuo Ashizawa and Thomas A Cooper and Juan Botas},
  journal={Human molecular genetics},
  year={2006},
  volume={15 13},
  pages={2138-45}
}
Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder caused by a CTG expansion in the 3' UTR of the dystrophia myotonica protein kinase (DMPK) gene. It has been hypothesized that the pathogenesis in DM1 is triggered by a toxic gain of function of the expanded DMPK RNA. This expanded RNA is retained in nuclear foci where it sequesters and induces alterations in the levels of RNA-binding proteins (RNA-BP). To model DM1 and study the implication of RNA-BP in CUG-induced toxicity, we have… CONTINUE READING

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