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Animal Models for Retinitis Pigmentosa

Animal Models for Retinitis Pigmentosa

Animal models are critical for understanding retinitis pigmentosa (RP) mechanisms and testing potential therapeutic approaches. Our company offers a range of services for RP animal modeling, including the generation of transgenic and knockout animal models and the characterization of model phenotypes. Our team of experienced scientists has extensive expertise in RP research and can help you design and execute animal modeling experiments. With our expertise, technology, and collaborative approach, we are well-positioned to support your research needs in RP.

Background

RP is a rare genetic disease that affects the retina. It is typically caused by a series of gene mutations or deletions leading to the gradual degeneration of a number of photoreceptor cells. Its clinical manifestations are usually night blindness, accompanied by progressive visual field defects, fundus pigmentation, and significant abnormalities on the retinal electroretinogram. RP inheritance patterns include autosomal dominant, autosomal recessive, X-linked, double gene, and mitochondrial patterns. RP is a common form of retinal degeneration (RD). A number of animal models of RD are available, including mice, rats, rabbits, pigs, zebrafish, and nonhuman primates. The study of these animal models can lead to a better understanding of the disease pathology and help develop therapeutic strategies to cure or slow down RD.

Fig. 1 Retinal degeneration in P347L rats.

Fig. 1 Retinal degeneration in P347L rats. (Inoue C, et al., 2021)

Disease Modeling Services

We offer our clients a wide range of RP animal models including natural and transgenic animal models. We use cutting-edge technology and techniques for the generation and characterization of RP animal models, such as CRISPR/Cas9-mediated genome editing, which allows for precise and efficient gene editing. The animal models we offer include:

  • Canine models
    We provide our clients with canine models of naturally occurring hereditary retinal degeneration to facilitate experiments difficult to perform in other organisms. Canine pedigrees have tremendous potential for genetic mapping, especially when well-characterized canine models are used for the analysis and treatment of corresponding human diseases. We provide canine models with mutations in the cGMP-PDEb gene, RHO gene, RPGR gene, or RPGRIP1 gene.
  • Cat models
    Cat globes are similar in physiology and anatomy to human globes, making them an effective model for studying transplantation, gene therapy, and drug therapy. We provide cat models including the rdAc (late-onset photoreceptor degeneration) cat model and the Rdy model.
  • Rodent models
    Rodent models offer low cost, relatively rapid disease progression, and the ability to manipulate genetics. We offer a variety of rodent models for our clients including the rd10 mouse model, transgenic Rpe65–/– mouse model, RCS rat model, and transgenic P23H rat model.
  • Pig models
    The pig eye is similar in size to the human eye and has a similar number and distribution of rod and cone cells. We provide our clients with mutant rhodopsin transgenic pigs as models for RP research. The mutations are P347L and P347S, which cause autosomal dominant retinitis pigmentosa (ADRP) in humans.

Characterization of Model Phenotypes

We also offer services for the characterization of RP animal models to assess the effects of genetic mutations on retinal structure and function, as well as on visual behavior. Our services include:

  • Ophthalmic evaluation and fundic imaging
  • Confocal scanning laser ophthalmoscopy and spectral domain optical coherence tomography
  • Electroretinography
  • Vision testing
  • Tissue processing and sectioning
  • Immunohistochemistry
  • Imaging and quantification of immunolabeled retinal sections

We work closely with our clients to design and generate animal models that meet their specific research needs. We keep abreast of the latest research in the field and are committed to providing the highest quality service to accelerate the identification of causative mechanisms in RD and the development of treatments to enhance photoreceptor survival and cure RD through gene replacement. If you are interested in our animal modeling services, please contact us for more information.

Reference

  • Inoue, C.; et al. A rat model for retinitis pigmentosa with rapid retinal degeneration enables drug evaluation in vivo. Biological Procedures Online, 2021, 23(1): 1-11.

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.

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