- Zebrafish Germ Cell Tumor Models
- Zebrafish Intestinal Cancer Models
- Zebrafish Intrahepatic Cholangiocarcinoma Models
- Zebrafish Liver Cancer Models
- Zebrafish Melanoma Models
- Zebrafish Neurofibromatosis Type 1 Models
- Zebrafish Pancreatic Cancer Models
- Zebrafish Retinoblastoma Models
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- Zebrafish Thyroid Cancer Models
Zebrafish Retinitis Pigmentosa Models
The wide variety of photoreceptor degenerative diseases present challenges to preventing or reversing vision loss. Retinitis pigmentosa (RP), also known as erythrodystrophy, is a group of rare genetic disorders characterized by decreased night vision and peripheral vision loss due to progressive photoreceptor cell death and photoreceptor dysfunction. The condition also changes the way the retina responds to light, causing initial vision problems in addition to gradual loss of vision over time. So far, there is no single treatment for RP, although researchers are looking for ways to slow the rate of vision loss.
The most common cause of human autosomal RP is a mutation in the rod-specific rhodopsin (RHO) gene. Recently, various RHO mutant zebrafish models have been established associated with dominant or recessive RP, with progressive rod degeneration. Importantly, the zebrafish cone photoreceptors were not affected by the RHO mutation, which is consistent with the characteristics of human RP caused by the RHO mutation. Due to the similarities in retinal anatomy and function between zebrafish and humans, the zebrafish model has gradually become the main model for studying photoreceptor development and disease.
Fig.1 Disruption of the N-linked glycosylation sequence in rhofl10 and rhofl7 leads to rod degeneration.
Our Zebrafish Retinitis Pigmentosa Models
Creative Biogene uses forward genetic screens for zebrafish visual behavior and light response research to identify genetic mutations associated with retinal degeneration and to build genetic models to study pathology. We offer a variety of transgenic zebrafish models that mimic various phenotypes of human RP. These zebrafish models of retinal degeneration provide an opportunity to not only characterize the disease, but also determine the mechanisms of photoreceptor regeneration. Overall, we are committed to helping you understand the molecular cues of central retinal development and regeneration most relevant to human vision through the zebrafish.
Table 1 Zebrafish models of retinitis pigmentosa-like diseases.
| Gene | Photoreceptor Features |
|---|---|
| adipor1 | Decrease in rod photoreceptors |
| cerkl | Photoreceptor functional defects at 7 dpf. Rod OS defects at 3 months, cone OS defects at 7 months. Notable thinning of the photoreceptor layer and cell death by 12 months |
| dact2 | Photoreceptor disc membrane disarrangement at 5 dpf |
| eys | Progressive photoreceptor loss; cone degeneration at 6 months, rod degeneration at 14 months |
| her9 | Decrease in rod photoreceptors at 5 dpf. Few double cones with short OSs at 12 dpf |
| kif3b | Delayed OS development. Rapid rod degeneration by 5 dpf |
| myo7aa | Decreased photoreceptor function at 5dpf. Reduced rods at 8 dpf |
| poc1 | Decrease length of photoreceptor OSs at 4 dpf |
| prom1 | Decrease in cone photoreceptors at 7 dpf. Longer rod Oss. Delayed development of OSs |
| prpf31 | Decreased in neuronal precursors and mature neurons at both 48 and 60 hpf |
| rho | Rod loss observed at 6 dpf. Degeneration continues into adulthood |
| rp1l1 | Rod dysfunction at 6 months. Subretinal drusenoid deposits at 11 months. Photoreceptor loss at 12 months |
| rp2 | Photoreceptor functional defects at 7 dpf. Short rod OSs at 2 months; cone OS defects at 4 months; significant rod OS loss and decreased cone OSs by 7 months |
| rpgrip1 | No rod OSs at 5 dpf. Cone dysfunction at 7 dpf. Severe rod degeneration by 3 months, followed by cone degeneration. Degeneration of most photoreceptors by 23 months |
| slc7a14 | Decreased photoreceptor function at 5 dpf. Reduced rod photoreceptors and peripheral RPE at 5 dpf |
| SNRNP200 | Photoreceptors loss at 3 dpf |
| ush2a | Decreased photoreceptor function at 5–7 dpf and increased photoreceptor apoptosis at 8 dpf. Notable rod OS degeneration at 12 months, cone OS degeneration at 20 months |
References
- Zelinka CP, Sotolongo-Lopez M, Fadool JM. Targeted disruption of the endogenous zebrafish rhodopsin locus as models of rapid rod photoreceptor degeneration. Mol Vis. 2018, 24:587-602.
- Nakao T, et al. The role of mislocalized phototransduction in photoreceptor cell death of retinitis pigmentosa. PLoS One. 2012, 7(4): e32472.
- Hong Y, Luo Y. Zebrafish Model in Ophthalmology to Study Disease Mechanism and Drug Discovery. Pharmaceuticals (Basel). 2021, 14(8):716.
For research use only. Not intended for any clinical use.
