July 2019
Volume 60, Issue 9
Free
ARVO Annual Meeting Abstract  |   July 2019
AAV-induced re-expression of Dp71 in retinal Müller glial cells recovers electroretinographic responses in Dp71-null mice
Author Affiliations & Notes
  • Mirella Telles Salgueiro Barboni
    Experimental Psychology, University of Sao Paulo, S�o Paulo, S�o Paulo, Brazil
    Ophthalmology, Semmelweis University, Budapest, Hungary
  • Cyrille Vaillend
    Neuroscience Paris-Saclay Institute (Neuro-PSI), Université Paris Sud, CNRS, Université Paris Saclay, France
  • Anneka Joachimsthaler
    University Hospital Erlangen, Erlangen, Germany
  • Andre Liber
    Experimental Psychology, University of Sao Paulo, S�o Paulo, S�o Paulo, Brazil
  • Hanen Khabou
    Institut de la Vision, Paris, France
  • Michel J Roux
    University of Strasbourg, France
  • lucile vignaud
    Institut de la Vision, Paris, France
  • Deniz Dalkara
    Institut de la Vision, Paris, France
  • Xavier Guillonneau
    Institut de la Vision, Paris, France
  • Dora Ventura
    Experimental Psychology, University of Sao Paulo, S�o Paulo, S�o Paulo, Brazil
  • Alvaro Rendon
    Institut de la Vision, Paris, France
  • Jan J Kremers
    University Hospital Erlangen, Erlangen, Germany
  • Footnotes
    Commercial Relationships   Mirella Barboni, None; Cyrille Vaillend, None; Anneka Joachimsthaler, None; Andre Liber, None; Hanen Khabou, None; Michel Roux, None; lucile vignaud, None; Deniz Dalkara, None; Xavier Guillonneau, None; Dora Ventura, None; Alvaro Rendon, None; Jan Kremers, None
  • Footnotes
    Support  This study was supported by Sao Paulo Research Foundation (FAPESP grant numbers 2016/22007-5 to MTSB; 2016/04538-3 and 2014/26818-2 to DFV), National Council for Scientific and Technological Development (CNPq grant number 404239/2016-1 to MTSB), Agence Nationale de la Recherche (ANR, France, grant ANR-14-CE13-0037-01 to CV), Centre National de la Recherche Scientifique (CNRS, France) and University Paris-Sud (France). DFV is a CNPq 1A productivity fellow. MTSB was a fellow of Campus France (N° 931824L).
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4240. doi:
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      Mirella Telles Salgueiro Barboni, Cyrille Vaillend, Anneka Joachimsthaler, Andre Liber, Hanen Khabou, Michel J Roux, lucile vignaud, Deniz Dalkara, Xavier Guillonneau, Dora Ventura, Alvaro Rendon, Jan J Kremers; AAV-induced re-expression of Dp71 in retinal Müller glial cells recovers electroretinographic responses in Dp71-null mice. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4240.

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

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Abstract

Purpose : ERG b-waves are associated with retinal bipolar cells activity but a contribution of Müller glial cells (MGCs) is still debated. Targeted disruption of the glial dystrophin Dp71 in mice and selective rescue of Dp71 function in MGCs may provide insights into the contribution of these cells to the ERG.

Methods : We recorded ERG responses to scotopic and photopic flashes (1), to mesopic and photopic 4 Hz rapid-on and –off sawtoth modulation (2) and to sine wave modulation at different temporal frequencies (3) from Dp71-null mice before and after intravitreal injections of ShH10-GFP-2A-Dp71 (an AAV designed to selectively target MGCs; Vacca et al. 2016 Hum. Mol. Genet. 25:3070-3079) and in wildtype (WT) littermates.

Results : Before treatment, Dp71-null mice displayed reduced b-waves in scotopic and photopic flash ERGs (1) and smaller response amplitudes to photopic rapid-on sawteeth (2) and to sine wave flicker responses at low temporal frequencies (3). These responses were completely recovered 12 weeks after ShH10-GFP-2A-Dp71 injections and reached the same level as found in the WT mice.

Conclusions : ERG b-waves and other components involving ON-bipolar and cone pathways are influenced by Müller glial cells' integrity. Deficits of retinal function can be successfully reversed by gene therapeutic intervention with ShH10-GFP-2A-Dp71 mediating re-expression of Dp71.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

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