A Light-Deprivation Mouse Model Potentially for Studying the Complete Congenital Stationary Night Blindness

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.

• Complete Congenital Stationary Night Blindness
• Light-Deprivation
• Electroretinogram
• Oscillatory Potentials

Acknowledgements

This study was supported by the grants from the Natural Science Foundation of Zhejiang Province of China (No.

Table 1

Comparison in ERG implicit time and amplitude between the control and light-deprivation mice

		Implicit time (ms)		Amplitude (μV)
Item	Wave	Control (n = 17)	Light deprivation (n = 15)	Control (n = 17)	Light deprivation (n = 15)
Scot-ERG	a	23.82 ± 1.41	22.82 ± 2.41	7.99 ± 1.56	7.83 ± 2.61
	b	82.53 ± 2.70	87.55 ± 2.48	241.38 ± 21.71	158.88 ± 20.35**
Max-ERG	a	14.53 ± 0.40	16.73 ± 0.47**	175.58 ± 14.24	138.30 ± 27.18
	b	36.47 ± 3.12	44.18 ± 3.23	441.65 ± 37.43	271.00 ± 36.07**
Ops	P1	25.35 ± 0.51	25.64 ± 0.74	175.85 ± 23.05	65.05 ± 11.10***
Phot-ERG	a	15.86 ± 0.93	15.45 ± 0.93	11.07 ± 1.94	7.73 ± 1.36
	b	43.50 ± 1.40	43.18 ± 1.99	86.18 ± 10.70	67.34 ± 10.77
30-Hz-flicker	P2	51.00 ± 3.94	47.18 ± 3.45	19.90 ± 1.44	14.96 ± 2.01

Values indicate mean ± SE. Individual samples t-test. ^*P < 0.05, ^**P < 0.01, ^***P < 0.001.

LQ14H120003), the National Natural Science Foundation for Young Scientists of China (No. 81301117), the Natural Science Foundation of China (No. 81470660), and the Wenzhou Science and Technology Plans (No. Y20140139). The research leading to these results has also received funding from the European Union Seventh Framework Programme (FP7/2007-2013) under grant agreement n° 604102 (HBP).

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