%0 Journal Article
%T Properties of Flicker ERGs in Rat Models with Retinal Degeneration
%A Jing An
%A Qun Guo
%A Li Li
%A Zuoming Zhang
%J ISRN Ophthalmology
%D 2012
%R 10.5402/2012/346297
%X Purpose. To describe the characteristics of rod and cone functions in rat models for congenital stationary night blindness (CSNB) and retinal cone dysfunction (RCD). Methods. Rod and cone function were isolated by recording the rod-/cone-driven flicker and blue light flicker electroretinograms (ERGs). Results. During dark adaptation, the amplitudes of flicker ERGs in CSNB rats were lower than those in control rats; the responses of RCD rats were similar to control rats. During light adaptation, the amplitudes of flicker ERGs in CSNB rats were reduced; whereas the responses of RCD rats were not detected. Blue flicker ERGs were not observed in CSNB rats at lower frequencies. The cone driven critical flicker frequencies (CFFs) in control rats were 62£¿Hz. The rod driven CFF of RCD rats was 20£¿Hz; whereas the rod-/cone-driven CFF of CSNB rats both were about 25£¿Hz. Conclusions. The function of the rod system was damaged completely, the cones were the source of vision in CSNB rats. Rod system function is excellent in RCD rat. The rods of albinism rats are sensitive to frequencies less than 20£¿Hz; whereas the cones are sensitive to frequencies up to 62£¿Hz. 1. Introduction Inherited retinal degeneration disorder is one of the most serious diseases that cause blindness in humans. Gene mutations are expressed in photoreceptors, bipolar cells, or other part of the retina, which can cause various changes in rod and cone function. The loss of rod or cone path function or visual signal transduction results in serious eye disease in humans. However, the pathogenesis of retinal degeneration disorder is still not quite understood, and valid treatment is currently lacking. Clinically, the classification of retinal diseases mainly depends on electroretinograms (ERGs) to analyze the extent of damage to the cone and rod systems. Spontaneous generation animal models of retinal degeneration are important for studying gene mutation, protein function, and disease prevention, and cure. Congenital stationary night blindness (CSNB) is a nonprogressive retinal degeneration disease characterized by the loss of night vision with partially or completely absent rod function [1]. CSNB disease is transmitted via an autosomal recessive, autosomal dominant or X-linked mode of inheritance. Several groups have shown that the genetic heterogeneity of the X-linked CSNB (XLCSNB) is controlled by two different loci on the X chromosome. The NYX gene mutation gives rise to cCSNB (CSNB1), whereas the CACNA1F gene mutation leads to iCSNB (CSNB2) [2¨C7]. Retinal cone dysfunction (RCD) [8] is a retina
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