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Functional study in NSE-Hu-Bcl-2 transgenic mice: a model for retinal diseases starting in Müller cells
Cécile Péant1, André Dosso, Lorenza Eder-Colli
1Department of Basic Neuroscience, Medical School, University of Geneva, Geneva, Switzerland.
Abstract:
In NSE-Hu-Bcl-2 transgenic mice, line 71, retina undergoes early postnatal degeneration linked to the prior death of Müller cells. The purpose of this study was to complete the characterization of this retinal dysfunction by using electroretinographic (ERG) recordings in both scotopic and photopic conditions. Here, we showed that both rod and cone systems were profoundly affected in NSE-Hu-Bcl-2 transgenic mice as soon as 15 postnatal days in accordance with histological study performed previously.
Insights
NSE-Hu-Bcl-2 transgenic mice exhibit early retinal degeneration affecting both rod and cone systems. This dysfunction, observed at 15 postnatal days, is linked to Müller cell death.
Area of Science:
- Neuroscience
- Ophthalmology
- Genetics
Background:
- NSE-Hu-Bcl-2 transgenic mice, line 71, display progressive retinal degeneration.
- This degeneration is associated with early Müller cell death.
- Previous histological studies indicated significant retinal abnormalities.
Purpose of the Study:
- To fully characterize the retinal dysfunction in NSE-Hu-Bcl-2 transgenic mice.
- To assess the functional impact on rod and cone photoreceptor systems.
- To correlate electroretinographic findings with prior histological data.
Main Methods:
- Electroretinographic (ERG) recordings were performed.
- Recordings assessed both scotopic (rod-mediated) and photopic (cone-mediated) vision.
- Evaluations were conducted in NSE-Hu-Bcl-2 transgenic mice at 15 postnatal days.
Main Results:
- Both rod and cone photoreceptor systems were profoundly affected.
- Significant functional deficits were evident as early as 15 postnatal days.
- ERG results aligned with previous histological observations of retinal degeneration.
Conclusions:
- NSE-Hu-Bcl-2 transgenic mice demonstrate early and severe impairment of both rod and cone function.
- The observed retinal dysfunction is consistent with Müller cell loss.
- ERG is a valuable tool for characterizing visual pathway dysfunction in transgenic models.
