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Spatial vision and c-wave are affected in mouse models of human autosomal dominant retinitis pigmentosa caused by PRPF31 mutations

Session Details

Session Title: New Drug Treatment

Session Date/Time: Saturday 13/09/2014 | 14:30-16:30

Paper Time: 15:42

Venue: Boulevard F

First Author: : A.M.Aramburu del Boz SPAIN

Co Author(s): :    F.J. Díaz Corrales   B. de la Cerda Haynes   L. Valdés Sánchez   D. Rodriguez Martínez   S.S. Bhattacharya  

Abstract Details

Purpose:

To describe the optomotor test response and c-wave amplitude of electroretinogram in mouse models of autosomal dominant Retinitis Pigmentosa (adRP) associated to PRPF31 gene mutation.

Setting:

CABIMER (Andalucian Centre of Molecular Biology and Regenerative Medicine), Seville, Spain

Methods:

Two gene-target mouse models have been generated to study the physiopathology of the adRP linked to PRPF31 mutations. However, the characterization of Prpf31A216P/+ knockin (KI) and Prpf31+/- knockout (KO) mutant mice have not showed a clear degenerative phenotype. Only, it has been described alterations of the retinal pigment epithelium (RPE) in the Prpf31+/- mice at one year of age. Optomotor test evaluates the optokinetic reflex functionality of the mouse. This reflex is originated in the retina and is a good indicator of the retinal visual function of the mouse. On the other hand, the electroretinogram (ERG) c-wave is originated specifically in the RPE, and the amplitude and frequency of this ERG wave can be used to assess the functional integrity of RPE cells. To date, neither optomotor test nor c-wave have been evaluated in the Prpf31 KI and KO mutant mice. For those reasons, we deeply evaluate the optomor responses and the c-wave in those mutant mice. Optomotor test was performed in young and adult mice at different spatial frequencies and at three different levels of contrast sensitivity to evaluate their visual function and thresholds. Standard ERG recording and the c-wave as well as funduscopy were also performed.

Results:

We demonstrated that visual acuity as well as contrast sensitivity perception and c-wave were significantly affected in KI and KO mice. The visual function was more affected in the adult mutants, even at 6 months of age. At this age, drusen-like deposits were observed in the entire retina at the funduscopy examination. At this age, however, photoreceptors seem normal.

Conclusions:

Even when the characterization of the KI and KO models has not showed a clear phenotype, our results show that retinal function is affected in Prpf31 mutant mice, even before photoreceptor degeneration can be detected. These results might be useful in preclinical studies to evaluate the efficacy of advanced therapies using these mouse models of adRP.

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