Name: Rpgr KO Mouse
Brand: 싸이아젠(Cyagen)
SKU: C001823

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Rpgr KO Mouse

제품명

Rpgr KO Mouse

제품 ID

C001823

품종 계통

C57BL/6JCya-Rpgr^em1/Cya

Backgroud

C57BL/6JCya

상태

이 마우스 계통을 논문에서 사용할 경우, “Rpgr KO Mouse (카탈로그 번호 C001823)은 Cyagen에서 구입하였습니다.”라고 명시해 주시기 바랍니다.

Disease Animal Models

구매 가능한 제품 종류

연령

Genotype

성별

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가격 문의

Disease Animal Models

기본 정보

검증 데이터

The Rpgr KO mouse is a gene knockout model created using gene-editing techniques to knock out the coding sequence of the Rpgr gene (the homolog of the human RPGR gene) in mice. This model can be used to study the pathogenic mechanisms of retinal diseases like X-linked retinitis pigmentosa (XLRP) and X-linked cone-rod dystrophy (CORDX1), providing a research basis for developing related therapeutic interventions.

Reference

Awadh Hashem S, Georgiou M, Ali RR, Michaelides M. RPGR-Related Retinopathy: Clinical Features, Molecular Genetics, and Gene Replacement Therapy. Cold Spring Harb Perspect Med. 2023 Nov 1;13(11):a041280.

Lam BL, Scholl HPN, Doub D, Sperling M, Hashim M, Li N. A SYSTEMATIC LITERATURE REVIEW OF DISEASE PROGRESSION REPORTED IN RPGR -ASSOCIATED X-LINKED RETINITIS PIGMENTOSA. Retina. 2024 Jan 1;44(1):1-9.

Pechnikova NA, Poimenidou M, Iliadis I, Zafeiriou-Chatziefraimidou M, Iaremenko AV, Yaremenko TV, Domvri K, Yaremenko AV. Pre-Clinical and Clinical Advances in Gene Therapy of X-Linked Retinitis Pigmentosa: Hope on the Horizon. J Clin Med. 2025 Jan 29;14(3):898.

변형 전략

The mouse Rpgr gene in mice consists of 18 exons, with the start codon in exon 1 and the stop codon in exon 18. This strain was created by gene-editing techniques that knocked out the region spanning exons 1~18.

Figure 1. Diagram of the gene editing strategy for the generation of Rpgr KO mice.

응용 분야

Research on X-linked retinitis pigmentosa (XLRP);

Research on X-linked cone-rod dystrophy (CORDX1);

Research on other retinal diseases.

검증 데이터

1. Fundus morphology & OCT

Figure 2. Fundus morphology and OCT results of WT and Rpgr KO mice at 6 weeks of age.

Figure 3. Fundus morphology and OCT results of WT and Rpgr KO mice at 3 months of age.

Figure 4. Fundus morphology and OCT results of WT and Rpgr KO mice at 6 months of age.

Figure 5. Fundus morphology and OCT results of WT and Rpgr KO mice at 10 months of age.

Figure 6. Fundus morphology and OCT results of WT and Rpgr KO mice at 15 months of age.

Figure 7. OCT imaging of Rpgr KO mice at different weeks of age revealed that, similar to WT mice, most retinal layers remained relatively intact at the imaging time point. Compared to WT controls, Rpgr KO mice exhibited mild but not significant retinal thinning and progressed at a very slow rate. However, four hyper-reflective bands (ORRB1–4) were clearly distinguishable in the outer retinas of WT mice, these bands were indistinct in Rpgr KO mice. In Rpgr KO mice, the demarcation of ORRB2 progressively thickened, with complete obliteration of the boundary between ORRB2 and ORRB3 particularly beyond 6 months of age.

2. Magnified images selected from OCT results

Figure 8. Magnified images selected from OCT results of WT and Rpgr KO mice. There are four distinguishable hyper-reflective bands in the outer retina, namely outer retina reflective bands (ORRBs) 1–4, which correspond to the outer limiting membrane, ellipsoid zone (EZ), interdigitation zone (IZ), and retinal pigment epithelium (RPE)/Bruch’s membrane complex, respectively. Four hyper-reflective bands (ORRB1–4) were clearly distinguishable in the outer retinas of WT mice. However, a common band change was observed in Rpgr KO mice: the boundary between ORRB2 and ORRB3 was almost invisible and the two bands had merged by the time the mice were 6 months old.

3. Electroretinogram (ERG)

Figure 9. Electroretinogram (ERG) detection results of WT and Rpgr KO mice at 6 weeks of age. At 6 weeks of age, the amplitudes of the a-wave and b-wave in both scotopic and photopic electroretinograms (ERGs) of Rpgr KO mice were almost identical to those of WT mice.

Figure 10. Electroretinogram (ERG) detection results of WT and Rpgr KO mice at 6 months of age. By 6 months of age, the amplitudes of the a-wave and b-wave in scotopic ERG recordings of Rpgr KO mice had begun to decrease gradually.

Figure 11. Electroretinogram (ERG) detection results of WT and Rpgr KO mice at 10 months of age. By 10 months of age, the amplitudes of the a-wave and b-wave in scotopic ERG recordings of Rpgr KO mice had begun to decrease gradually.

Figure 12. Electroretinogram (ERG) detection results of WT and Rpgr KO mice at 15 months of age. By 15 months of age, the a-wave and b-wave amplitudes in the scotopic ERG recordings of Rpgr KO mice were significantly lower than in WT mice.

4. Retinal immunofluorescence staining results

Figure 13. Retinal immunofluorescence staining results of WT and Rpgr KO mice at 8 weeks of age. The retinal sections of WT and Rpgr KO mice were evaluated histologically. At 8 weeks of age, compared with WT, the retinal structure and rhodopsin & PNA protein expression in Rpgr KO mice were normal. Although retinal cell loss was not apparent in young Rpgr KO retinas, staining for retinal cell markers, which are downregulated during retinal degeneration, indicated that degenerative changes were already underway.

Figure 14. Retinal immunofluorescence staining results of WT and Rpgr KO mice at 15 months of age. In 15-month-old Rpgr KO mice, rhodopsin and PNA expression decreased dramatically, and only sparse PNA staining was visible, which was consistent with the decreased outer-segment integrity and protein stability.

Rpgr KO Mouse

기본 정보

검증 데이터

관련 자료

유전자명

유전자 별칭

NCBI ID

염색체

MGI ID

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Datasheet

품종 계통 설명

Reference

변형 전략

응용 분야

1. Fundus morphology & OCT

2. Magnified images selected from OCT results

3. Electroretinogram (ERG)

4. Retinal immunofluorescence staining results

관련 제품

리소스