December 2002
Volume 43, Issue 13
Free
ARVO Annual Meeting Abstract  |   December 2002
Improvement of Neuronal Visual Responses in the Superior Colliculus in Retinal Degeneration Slow Mice by Gene Therapy
Author Affiliations & Notes
  • FC Schlichtenbrede
    Molecular Genetics
    Instit of Ophthalmology London United Kingdom
  • L da Cruz
    Molecular Genetics
    Instit of Ophthalmology London United Kingdom
  • C Stephens
    Molecular Genetics
    Instit of Ophthalmology London United Kingdom
  • JW B Bainbridge
    Molecular Genetics
    Instit of Ophthalmology London United Kingdom
  • T Salt
    Visual Science
    Instit of Ophthalmology London United Kingdom
  • RR Ali
    Molecular Genetics
    Instit of Ophthalmology London United Kingdom
  • Footnotes
    Commercial Relationships   F.C. Schlichtenbrede, None; L. da Cruz, None; C. Stephens, None; J.W.B. Bainbridge, None; T. Salt, None; R.R. Ali, None. Grant Identification: Support: EU Grant
Investigative Ophthalmology & Visual Science December 2002, Vol.43, 4603. doi:
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      FC Schlichtenbrede, L da Cruz, C Stephens, JW B Bainbridge, T Salt, RR Ali; Improvement of Neuronal Visual Responses in the Superior Colliculus in Retinal Degeneration Slow Mice by Gene Therapy . Invest. Ophthalmol. Vis. Sci. 2002;43(13):4603.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Abstract: : Purpose: The retinal degeneration slow (rds or Prph2Rd2/Rd2) mouse, a model of recessive retinitis pigmentosa, lacks a functional gene encoding peripherin 2, which is required for the formation of photoreceptor outer segment discs. We have previously demonstrated restoration of photoreceptor ultrastructure and function by adeno-associated virus (rAAV) mediated gene transfer of peripherin 2. Here we have extended our assessment to central visual neuronal responses in order to show an improvement of central visual function. Methods: rAAV encoding the Prph2-transgene was injected subretinally into one eye of 10 rds-mice on postnatal day10. Three to 5 weeks later electroretinograms (ERGs) were performed to demonstrate retinal rescue. Three mice were used to record central visual responses of neurons in the superior colliculus. Visual stimuli were presented on a CRT screen as moving bars or flashing spots of varying luminance. For both methods the untreated contralateral eye served as control. Results: Simultaneous ERGs of right and left eyes showed a substantial increase in b-wave amplitude and improvement in waveform on the treated side. This effect was first noted three weeks post injection and persisted for at least six weeks, showing good retinal responses on the treated side and low residual responses contralaterally. Recordings from visually responsive neurons in the superior colliculus showed clear responses to stimuli and a significant reduction in stimulus threshold as compared to recordings on the untreated contralateral side. Conclusions: These findings suggest that gene replacement therapy not only restores photoreceptor morphology and function in the rds mouse, but also improves central visual responses.

Keywords: 419 gene transfer/gene therapy • 562 retinal degenerations: hereditary • 518 photoreceptors: visual performance 
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