March 2012
Volume 53, Issue 14
ARVO Annual Meeting Abstract  |   March 2012
In Vivo Phenotyping Of A Usher 1 Mouse Model
Author Affiliations & Notes
  • Alix Trouillet
    Institut de la Vision, Paris, France
  • Julie Degardin
    Institut de la Vision, Paris, France
  • Amrit Estivalet
    Institut de la Vision, Paris, France
  • Iman Sahly
    Institut de la Vision, Paris, France
  • Christine Petit
    Institut Pasteur, Paris, France
  • Serge Picaud
    Institut de la Vision, Paris, France
  • Footnotes
    Commercial Relationships  Alix Trouillet, None; Julie Degardin, None; Amrit Estivalet, None; Iman Sahly, None; Christine Petit, None; Serge Picaud, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science March 2012, Vol.53, 2450. doi:
  • Views
  • Share
  • Tools
    • Alerts
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      Alix Trouillet, Julie Degardin, Amrit Estivalet, Iman Sahly, Christine Petit, Serge Picaud; In Vivo Phenotyping Of A Usher 1 Mouse Model. Invest. Ophthalmol. Vis. Sci. 2012;53(14):2450.

      Download citation file:

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

  • Supplements

Purpose: : Usher syndrome (US) is a recessively inherited disorder combining retinitis pigmentosa and a sensorineural hearing loss. The molecular mechanisms underlying the visual impairment are poorly understood and characterized. New Usher mouse models were recently generated to investigate the disease physiopathology and develop gene therapy. The purpose of this study was to analyse at a functional and a morphological level the retinal phenotypes of a mouse model of Usher Syndrome I ( SANS protein).

Methods: : In vivo measurements were achieved on mice deficient for Ush1G gene and compared to a control group. All the subjects were 18 months years old at the time of the experiment. The functional analysis was performed with a xenon lamp electroretinogram for measuring retinal responses at different light intensities in scotopic, photopic and flicker conditions. The retinal morphology was analyzed by ocular coherence tomography (OCT) in all quadrants of the eye.

Results: : High resolution OCT images did show any difference in the retinal thickness between US1G and control groups, assuming there is no detectable photoreceptor degeneration. In electronretinogram measurement under both scotopic, photopic and flicker light conditions, no change in amplitude or latency could be detected.

Conclusions: : This study indicate that no photoreceptor degeneration can be detected in vivo on Usher1 mice, which will hamper the development of a therapy on the murine model. Future studies will investigate at the histological level if cellular and molecular changes can be detected in the deficient mice.

Keywords: retinal degenerations: hereditary • pathobiology 

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.