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Japanese Journal of Ophthalmology
Published in: Japanese Journal of Ophthalmology 5/2015

01-09-2015 | Laboratory Investigation

Functional and morphological study of retinal photoreceptor cell degeneration in transgenic rabbits with a Pro347Leu rhodopsin mutation

Authors: Ken Asakawa, Hitoshi Ishikawa, Shigekazu Uga, Kimiyo Mashimo, Kimiya Shimizu, Mineo Kondo, Hiroko Terasaki

Published in: Japanese Journal of Ophthalmology | Issue 5/2015

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Abstract

Purpose

To investigate the process of retinal degeneration by analyzing the functional and morphological findings in transgenic rabbits with a Pro347Leu rhodopsin mutation.

Methods

Wild-type (WT) and transgenic (Tg) rabbits at ages 4, 8 and 12 months were used. We conducted functional evaluation by recording the changes in the pupil response to red and blue light stimulation and the amplitude of the electroretinography (ERG). Morphologically, rod and cone distribution was examined using light and electron microscopy. Immunostaining for the identification of retinal ganglion cells (RGCs) was also confirmed by injecting a TUJ-1 monoclonal antibody.

Results

Pupil constriction for infrared pupillography and the a- and b-waves for ERG in Tg rabbits decreased with increasing age; the differences were compared to the age-matched WT rabbits. The subnormal ERG in the Tg rabbits, especially the a-wave decrease and pupil constriction with a long latency time, was induced only during exposure to blue light stimulation at 12 months. Light and electron microscopic findings showed a progressive loss of photoreceptor cells over time manifesting by 8 months in the peripheral retina. Moreover, pyknotic nuclei of the outer nuclear layer in the center of the visual streak were observed. At 12 months, there was disappearance of the rods and ballooning degeneration of the cones. Some remaining RGCs had large cell bodies with long branching dendrites.

Conclusions

The changes in the pupil light response and amplitude of the ERG could be used to predict the state of retinal degeneration in the Tg rabbit.
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Metadata
Title
Functional and morphological study of retinal photoreceptor cell degeneration in transgenic rabbits with a Pro347Leu rhodopsin mutation
Authors
Ken Asakawa
Hitoshi Ishikawa
Shigekazu Uga
Kimiyo Mashimo
Kimiya Shimizu
Mineo Kondo
Hiroko Terasaki
Publication date
01-09-2015
Publisher
Springer Japan
Published in
Japanese Journal of Ophthalmology / Issue 5/2015
Print ISSN: 0021-5155
Electronic ISSN: 1613-2246
DOI
https://doi.org/10.1007/s10384-015-0400-6

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