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Customized Zebrafish Models of Retinitis Pigmentosa

The zebrafish model can well simulate various phenotypes of human RP and has invaluable value in identifying the pathogenic genes of RP. As one of the global leaders in the field of zebrafish eye disease research, Ace Therapeutics provides our customers with a series of zebrafish models of retinitis pigmentosa to facilitate the discovery and screening of RP drugs.

Zebrafish Models of Retinitis Pigmentosa

What Is Retinitis Pigmentosa?

Retinitis pigmentosa (RP) is a genetically based degenerative disease of the retina that affects approximately 1 in 4,000 people worldwide. It is characterized by decreased night vision and loss of peripheral vision due to progressive photoreceptor cell death and photoreceptor dysfunction. Unfortunately, for the vast majority of patients with this disease, there are currently no effective treatment options, highlighting the urgent need for effective and inexpensive RP therapies. The zebrafish is an ideal model for in vivo screening of therapeutic RP drugs because of its low cost of use, high fecundity, and ease of genetic manipulation. It could also facilitate RP drug discovery due to the rapid development of its visual system. Collectively, the zebrafish model promises to provide an excellent tool to gain insight into chronic diseases similar to RP.

Fig. 1. Expression of P23H Flag-tagged rhodopsin in adult zebrafish retina. (Santhanam A, et al., 2020)

Service Overview

Retinitis pigmentosa is a group of diseases that cause progressive retinal degeneration. These diseases are characterized by photoreceptor dysfunction with attenuation of retinal blood vessels and progressive cell death. Ace Therapeutics offers a variety of zebrafish models for this disease associated with dominant or recessive RP, represented by the RHO mutant zebrafish model. Importantly, cone photoreceptors in zebrafish are not affected by RHO mutants, which is consistent with the characteristics of human RP caused by RHO mutations.

Explore Ace Therapeutics' Zebrafish Models of Retinitis Pigmentosa

Ace Therapeutics researchers use forward mutagenesis screens and reverse genetics approaches to identify genetic mutations associated with retinal degeneration and isolate zebrafish phenotype models associated with RP. These models provide our clients with an effective system to study the molecular mechanisms involved in the development and regeneration of the central retina most relevant to human vision.

Importantly, our zebrafish model offers more possibilities for advancing retinal degeneration research. As well as characterizing the disease, it can determine the mechanism by which photoreceptor regeneration takes place. Compared to our rodent retinitis pigmentosa model, the zebrafish model also allows large-scale screening of phenotypic drugs, with lead compounds evaluated in a range of zebrafish models to identify compounds that are effective across species and RP models. Please contact our scientists to select the appropriate animal model to advance your retinitis pigmentosa project.

Ace Therapeutics provides the following zebrafish retinitis pigmentosa models to customers around the world, including but not limited to:

Tab.1. Ace Therapeutics' zebrafish models of retinitis pigmentosa.

Gene	Photoreceptor Features
adipor1	Decrease in rod photoreceptors.
cerkl	Photoreceptor functional defects at 7 dpf. Rod outer segment defects at 3 months, cone outer segment 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 outer segments at 12 dpf.
kif3b	Delayed outer segments development. Rapid rod degeneration by 5 dpf.
myo7aa	Decreased photoreceptor function at 5dpf. Reduced rods at 8 dpf.
poc1	Decrease length of photoreceptor outer segments at 4 dpf.
prom1	Decrease in cone photoreceptors at 7 dpf. Longer rod segments. Delayed development of outer segments.
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 outer segments at 2 months; cone outer segment defects at 4 months; significant rod outer segment loss and decreased cone outer segments by 7 months.
rpgrip1	No rod outer segments 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 outer segment degeneration at 12 months, cone outer segment degeneration at 20 months.

Ace Therapeutics aims to provide a powerful analytical tool to help our global customers understand the pathological features of retinal pigmentary diseases and the mechanisms of photoreceptor regeneration. If you are interested in our services or need more detailed information, please feel free to contact us. Our experienced scientists are ready to help you!

References

Santhanam A, Shihabeddin E, Atkinson JA, et al. A Zebrafish Model of Retinitis Pigmentosa Shows Continuous Degeneration and Regeneration of Rod Photoreceptors. Cells. 2020, 9(10):2242.
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.

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