Related Experiment Video
Updated: Feb 23, 2026
07:43
Subretinal Transplantation of Human Embryonic Stem Cell-Derived Retinal Tissue in a Feline Large Animal Model
Published on: August 5, 2021
2.4K
Central retinal preservation in rdAc cats.
Andrea Louise Minella1, Laurence Mireille Occelli1, Kristina Narfström2
1Department of Small Animal Clinical Sciences, College of Veterinary Medicine, Michigan State University, East Lansing, MI, USA.
Veterinary Ophthalmology
|September 1, 2017
Summary
Leber congenital amaurosis (LCA) in rdAc cats shows central retinal preservation and maintained ellipsoid zone (EZ) integrity, mirroring human disease patterns. This finding supports the use of rdAc cats as a model for studying LCA progression.
Area of Science:
- Ophthalmology
- Genetics
- Retinal Degeneration
Background:
- Leber congenital amaurosis (LCA) is a severe inherited retinal dystrophy.
- CEP290 mutations cause LCA, characterized by macular sparing in humans.
- Spectral domain-optical coherence tomography (SD-OCT) assesses retinal structure and disease progression.
Purpose of the Study:
- To investigate retinal degeneration patterns in rdAc cats with Cep290 mutations.
- To compare retinal structural changes in rdAc cats to human LCA patients.
- To evaluate the ellipsoid zone (EZ) integrity as a marker of degeneration in rdAc cats.
Main Methods:
- Utilized fundus imaging, confocal scanning laser ophthalmoscopy, and SD-OCT on 11 rdAc cats (6 months to 10 years).
- Measured photoreceptor (REC+) thickness in central and peripheral retinal regions.
- Assessed EZ integrity and quantified regional degeneration rates.
Main Results:
- RdAc cats exhibited progressive photoreceptor thinning across all retinal regions.
- Central retina (area centralis, visual streak) showed slower thinning rates compared to the mid-periphery.
- Ellipsoid zone (EZ) integrity was preserved centrally while degenerated in peripheral regions.
Conclusions:
- RdAc cats demonstrate central retinal preservation and maintained EZ integrity, consistent with human LCA findings.
- These findings validate the rdAc cat as a relevant animal model for CEP290-associated LCA.
- The study highlights EZ integrity as a key indicator of retinal function and disease progression in LCA models.
