Abstract
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.
Highlights
Inherited retinal degeneration disorder is one of the most serious diseases that cause blindness in humans
The amplitudes of the b-wave of the rod ERG responses were not detected in the Congenital stationary night blindness (CSNB) rats (Figure 1, right trace in the panel), standard combined ERG showed typical negative waveforms, and the cone response ERGs were markedly reduced in the CSNB rats
Cone-driven ERGs were not elicited in the retinal cone dysfunction (RCD) rats (Figure 1, middle trace in the panel)
Summary
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. 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
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
