Cone opsin determines the time course of cone photoreceptor degeneration in Leber congenital amaurosis.

@article{Zhang2011ConeOD,
  title={Cone opsin determines the time course of cone photoreceptor degeneration in Leber congenital amaurosis.},
  author={Tao Zhang and Ning Zhang and Wolfgang Baehr and Yingbin Fu},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2011},
  volume={108 21},
  pages={8879-84}
}
Mutations in RPE65 or lecithin-retinol acyltransferase (LRAT) disrupt 11-cis-retinal recycling and cause Leber congenital amaurosis (LCA), the most severe retinal dystrophy in early childhood. We used Lrat(-)(/-), a murine model for LCA, to investigate the mechanism of rapid cone degeneration. Although both M and S cone opsins mistrafficked as reported previously, mislocalized M-opsin was degraded whereas mislocalized S-opsin accumulated in Lrat(-)(/-) cones before the onset of massive ventral… CONTINUE READING
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Effect of gene therapy on visual function in Leber’s congenital amaurosis

  • JW Bainbridge
  • N Engl J Med
  • 2008

Safety and efficacy of gene transfer for Leber’s congenital amaurosis

  • AM Maguire
  • N Engl J Med
  • 2008

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