Abstract
Current rodent models of the complete congenital stationary night blindness (CSNB1) were time- consuming in breeding and validation, which makes it imperative to find a more “easily handle” animal model to broaden our understanding of this disorder. In the present study, a light-deprivation (LD) mouse model was made to validate whether it was a more “suitable” animal mode for investigating the pathogenesis of the CSNB1. Compared with controls, the LD mice exhibited a remarkable reduction in the amplitude of the dark-adapted electroretinogram (ERG) b-wave, the Max-ERG b-wave and also the oscillatory potentials (Ops), indicating an abnormal activity of rod bipolar cells in the retina. However, the ERG a-wave was relatively normal in the LD mice, which was quite consistent with what was confirmed in previously reported animal models of the CSNB1 and CSNB patients. Taken together, the LD mouse model showed CSNB1-like negative ERG responses as evidenced by the abnormal b-wave. Our study will provide a potentially useful animal model to decipher the pathogenesis of the CSNB1.
Highlights
The congenital stationary night blindness (CSNB) is a non-progressive disorder of the retina characterized by loss of night vision [1], which could be sub-classified as complete CSNB (CSNB1) and incomplete CSNB (CSNB2) [2]
The rodent model for this disorder was mainly the X-linked [3]-[6]. More or less, these rodent models were time-consuming in breeding and validation, which makes it imperative to find a more “ handle” animal model to broaden our understanding of the CSNB1
A plenty variety of animal models emerge aiming to investigate the pathogenesis of the CSNB1
Summary
The congenital stationary night blindness (CSNB) is a non-progressive disorder of the retina characterized by loss of night vision [1], which could be sub-classified as complete CSNB (CSNB1) and incomplete CSNB (CSNB2) [2]. Complete Congenital Stationary Night Blindness, Light-Deprivation, Electroretinogram, Oscillatory Potentials (2016) A Light-Deprivation Mouse Model Potentially for Studying the Complete Congenital Stationary Night Blindness.
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