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Animal Models for Stargardt's Disease

Animal models are essential for advancing our understanding of retinal degenerative diseases (RD) such as Stargardt's disease (STGD). Our company's animal models for STGD offer several advantages over other models currently available, including accurate pathology and the ability to test potential treatments in a preclinical setting. Our experienced team of scientists and technicians is committed to working closely with our clients to develop customized animal models that meet their specific research needs. We also provide a range of services, including phenotyping and data analysis, to ensure our clients' research is as efficient and effective as possible.

Background

Approximately 1 in 8,000-10,000 people are affected by STGD, a rare genetic eye disease that causes progressive vision loss. This disease typically occurs in children and young people and affects central vision, leading to blindness in severe cases. There are three recognized types of STGD, with type 1 (STGD1) being the most common (accounting for over 95% of the affected population) and caused by mutations in the Abca4 gene. There is currently no standard treatment method available.

Due to the complexity of STGD genetics and pathology, the development of accurate animal models has been challenging. Mice are currently widely used as animal models and provide valuable information for STGD research. However, mouse models are limited due to the significant differences between human and mouse eyes. Therefore, more appropriate animal models need to be developed to better understand the STGD mechanism and test novel potential therapeutic strategies.

Fig. 1 Comparison of retinal thickness among 9-month-old pigmented Abca4−/− mice before and seven days after BLI.

Fig. 1 Comparison of retinal thickness among 9-month-old pigmented Abca4−/− mice before and seven days after BLI. (Fang Y, et al., 2020)

Disease Modeling Services

We use a range of techniques, such as genetic modification and breeding, to develop customized animal models. Our models closely mimic human disease pathology, including retinal pigmented epithelium lipofuscin accumulation and the loss of photoreceptor cells. This makes our models ideal for studying disease progression and testing potential treatments. We provide animal models including:

  • STGD mouse model
    We generate pigmented Abca4−/− mice through a homozygous mutation in the Abca4 gene. These mice exhibit similar phenotypes to human diseases and produce the electrophysiological and morphological signs of retinitis pigmentosa.
    We provide another model for Stargardt's-like disease, the E_mut+/– mouse model, in which five base pairs are deleted in Elovl4 (elongation of very long chain fatty acids protein 4). This model is a viable option for developing new therapeutic approaches.
  • STGD pig model
    Porcine eyes have many similarities with human eyes in terms of size and retinal structure. Therefore, we are committed to helping clients generate STGD1 pig models. We used CRISPR/Cas9 technology to edit somatic cell nuclear transfer (SCNT) of primary fibroblasts to generate ABCA4 knockout (KO) pigs.

Characterization Services

Our models allow the testing of potential treatments in a preclinical setting, reducing clinical trial risk and cost. We work closely with our clients and provide professional characterization services. This is to ensure that their research is designed to meet regulatory requirements and has the most likely chance of success. Our services include, but are not limited to:

  • Blue-light illumination (BLI)
  • Electroretinography (ERG)
  • Fundus autofluorescence image acquisition
  • Image analysis
  • Optical coherence tomography (OCT)
  • Light microscopy, transmission electron microscopy (TEM), and fluorescence microscopy
  • Quantification of RPE and photoreceptor nuclei
  • Immunofluorescence staining
  • Quantification of bis retinoids

Our company is a leading provider of animal models for RD diseases. We have over 20 years of experience in the industry and have developed a range of suitable animal models for studying STGD pathology and evaluating treatments. Our high-quality images and reliable data analysis further efficiently optimize the drug development process and make us the best partner for your STGD drug development. If you are interested in our animal modeling services, please contact us for more information.

Reference

  • Fang, Y.; et al. Fundus autofluorescence, spectral-domain optical coherence tomography, and histology correlations in a Stargardt disease mouse model. The FASEB Journal, 2020, 34(3): 3693-3714.

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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