RS-1 Gene Delivery to an Adult Rs1h Knockout Mouse Model Restores ERG b-Wave with Reversal of the Electronegative Waveform of X-Linked Retinoschisis.

@article{Zeng2004RS1GD,
  title={RS-1 Gene Delivery to an Adult Rs1h Knockout Mouse Model Restores ERG b-Wave with Reversal of the Electronegative Waveform of X-Linked Retinoschisis.},
  author={Yong Zeng and Yuichiro Takada and Sten Kjellstrom and Kelaginamane T Hiriyanna and A. Tanikawa and Eric Wawrousek and Nizar Smaoui and Rafael Caruso and Ronald A. Bush and Paul A. Sieving},
  journal={Investigative ophthalmology & visual science},
  year={2004},
  volume={45 9},
  pages={3279-85}
}
PURPOSE To create and evaluate a mouse model of human X-linked juvenile retinoschisis (XLRS) and then investigate whether supplementing with the retinoschisin protein by gene delivery can reverse the abnormal "electronegative" electroretinogram (ERG) retinal response. METHODS An X-linked retinoschisis mouse (Rs1h-KO) model was created by substituting a neomycin resistance cassette for exon 1 and 1.6 kb of intron 1 of Rs1h, the murine orthologue of the human RS-1 gene. RS protein was evaluated… CONTINUE READING

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Prolonged recovery of retinal structure/function after gene therapy in an Rs1h-deficient mouse model of x-linked juvenile retinoschisis.

Molecular therapy : the journal of the American Society of Gene Therapy • 2005
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