Knockout Mouse Catalog | Cyagen APAC

Prph2-KO Mice

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Product Number:C001385

Genetic Background:C57BL/6J

Reproduction:Homozygote x Homozygote

Strain Description

The Peripherin 2 (PRPH2) gene encodes a protein that is a member of the transmembrane 4 superfamily, also known as the tetraspanin family, the majority of which are cell-surface proteins characterized by the presence of four hydrophobic structural domains that mediate signal transduction events and play important regulatory roles in cell development, activation, growth, and motility. Peripheral protein 2 is a cell surface glycoprotein found in the retinal optic rod and cone receptor cells of the eye. This protein is usually located in the limbic region of the outer segmental disc containing retinas, proteins responsible for initiating visual phototransduction at the reception of light signals. Peripheral protein 2 can act as an adhesion molecule involved in stabilizing and compacting the ocular outer segmental disc or maintaining the curvature of the limbus, and thus this protein is essential for the morphogenesis of the outer segmental disc and the transmission of light signals[1-2]. Defects in the PRPH2 gene have been associated with central and peripheral retinal degeneration, and common disorders include autosomal dominant retinitis pigmentosa (RP), Age-related macular degeneration (AMD), and macular dystrophies (MDs)[2].

This strain is a mouse Prph2 knockout model that uses gene editing technology to knock out the homolog of the human PRPH2 gene in mice. The deletion of Prph2 gene expression in mice leads to abnormalities in the morphogenesis of the outer segmental disc and the conduction of light signals, causing more delayed retinal degeneration (RD), and the progression of ocular retinal disease in this model is similar to that of mice carrying the RD2 spontaneous mutation in the mouse Prph2 gene[3], which is a class of animal models of delayed retinal degeneration.


The mouse Prph2 gene is located on chromosome 17, and exon 1-3 of this gene was knocked out using gene editing techniques.

● Retinitis Pigmentosa (RP) Research;

● Age-related Macular Degeneration (AMD) Research;

● Macular Dystrophy (MDs) Research.

1.Electroretinogram (ERG) testing

Figure 1. Electroretinogram (ERG) results of 11-week-old RD2 mice (Prph2) and wild-type mice (C57BL/6J). Compared with wild-type mice, the amplitudes of both a- and b-wave in scotopic and photopic ERG were significantly decreased in 11-week-old RD2 mice, with a more pronounced decrease in the dark scotopic ERG waveform, consistent with the clinical findings of significantly decreased or extinguished scotopic ERG in most retinitis pigmentosa diseases[4].


2.Optical coherence tomography (OCT) of the retina

Figure 2. Optical coherence tomography (OCT) results of 11-week-old RD2 mice (Prph2-KO) and wild-type mice (C57BL/6J). Compared to wild-type mice, RD2 mice showed abnormal retinal morphology with loss of the outer nuclear layer.


Retinal pathological evaluation of RD2 mice and wild-type mice by electroretinography (ERG) and optical coherence tomography (OCT) showed that RD2 mice exhibited retinal abnormalities compared with wild-type mice, and the amplitudes of both a- and b-wave in scotopic and photopic ERG were significantly decreased in 11-week-old RD2 mice, with the decrease in ERG waveforms being more pronounced.

In conclusion, RD2 mice are a delayed retinal degeneration model that can be used for subsequent studies of retinitis pigmentosa (RP) and other retinal diseases, providing a useful tool for the study of human diseases.



[1] Hartong DT, Berson EL, Dryja TP. Retinitis pigmentosa. Lancet. 2006 Nov 18;368(9549):1795-809.
[2] Farrar GJ, Kenna P, Jordan SA, Kumar-Singh R, Humphries MM, Sharp EM, Sheils DM, Humphries P. A three-base-pair deletion in the peripherin-RDS gene in one form of retinitis pigmentosa. Nature. 1991 Dec 12;354(6353):478-80.
[3] Schalken JJ, Janssen JJ, Sanyal S, Hawkins RK, de Grip WJ. Development and degeneration of retina in rds mutant mice: immunoassay of the rod visual pigment rhodopsin. Biochim Biophys Acta. 1990 Jan 29;1033(1):103-9.
[4] Hassan-Karimi H, Jafarzadehpur E, Blouri B, Hashemi H, Sadeghi AZ, Mirzajani A. Frequency Domain Electroretinography in Retinitis Pigmentosa versus Normal Eyes. J Ophthalmic Vis Res. 2012 Jan;7(1):34-8.