Progressive retinal degeneration and dysfunction in R6 Huntington's disease mice.

@article{Helmlinger2002ProgressiveRD,
  title={Progressive retinal degeneration and dysfunction in R6 Huntington's disease mice.},
  author={Dominique Helmlinger and Ga{\"e}l Yvert and Serge Picaud and Karine M{\'e}rienne and Jos{\'e} Alain Sahel and Jean-Louis Mandel and Didier Devys},
  journal={Human molecular genetics},
  year={2002},
  volume={11 26},
  pages={3351-9}
}
Huntington's disease (HD) and spinocerebellar ataxia type 7 (SCA7) belong to a group of progressive neurodegenerative diseases caused by polyglutamine (polyQ) expansions. SCA7 is the only one to display degeneration in the retina, a tissue usually spared in HD. We previously described a SCA7 transgenic retinal model expressing mutant full length ataxin-7 in rod photoreceptors. These mice develop a severe and characteristic retinopathy. We show here that R6 transgenic mice, which reproduce many… CONTINUE READING

Citations

Publications citing this paper.
Showing 1-10 of 33 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 31 references

Similar Papers

Loading similar papers…