Abstract
Studies utilizing large animal models of inherited retinal degeneration (IRD) have proven important in not only the development of translational therapeutic approaches, but also in improving our understanding of disease mechanisms. The dog is the predominant species utilized because spontaneous IRD is common in the canine pet population. Cats are also a source of spontaneous IRDs. Other large animal models with spontaneous IRDs include sheep, horses and non-human primates (NHP). The pig has also proven valuable due to the ease in which transgenic animals can be generated and work is ongoing to produce engineered models of other large animal species including NHP. These large animal models offer important advantages over the widely used laboratory rodent models. The globe size and dimensions more closely parallel those of humans and, most importantly, they have a retinal region of high cone density and denser photoreceptor packing for high acuity vision. Laboratory rodents lack such a retinal region and, as macular disease is a critical cause for vision loss in humans, having a comparable retinal region in model species is particularly important. This review will discuss several large animal models which have been used to study disease mechanisms relevant for the equivalent human IRD.
Highlights
Large animal models for inherited retinal degenerations (IRDs) have been identified within populations of dogs, cats, sheep, horses and, more recently, non-human primates (NHP)
IRDs that lead to visual impairment and blindness show considerable heterogeneity, where even similar disease phenotypes can result from mutations in a wide range of different genes
Laboratory rodents have been the workhorses for studying disease mechanisms because of the ease with which their genome can be engineered, the short reproductive time and relatively low costs of maintenance
Summary
Large animal models for inherited retinal degenerations (IRDs) have been identified within populations of dogs, cats, sheep, horses and, more recently, non-human primates (NHP). Cells 2020, 9, 882 germline transmission (see [5] for a review) or somatic gene knockout [6] Another major advantage of large animal models is the presence of a retinal region equivalent to the macula. The large animal model species considered here have an area centralis with high photoreceptor density, including, importantly, cones that are equivalent to the human macula [13]. The right layer (GCL), plexiform layer (IPL), inner nuclear (INL), outer plexifom layer outer panel depictsinner a schematic showing the genes detailedlayer in this review and their site of (OPL), expression, nuclear layer (ONL), external limiting membrane (ELM), photoreceptor innerlayer segment (IS), connecting grouped per biological process. Ganglion cell (GC), amacrine cell (AC), bipolar cell (BC), horizontal cell (HC), rod (R), cone (C)
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