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Retinal areas of specialization confer vertebrates with the ability to scrutinize corresponding regions of their visual field with greater resolution. A highly specialized area found in haplorhine primates (including humans) is the fovea centralis which is defined by a high density of cone photoreceptors connected individually to interneurons, and retinal ganglion cells (RGCs) that are offset to form a pit lacking retinal capillaries and inner retinal neurons at its center. In dogs, a local increase in RGC density is found in a topographically comparable retinal area defined as the area centralis. While the canine retina is devoid of a foveal pit, no detailed examination of the photoreceptors within the area centralis has been reported. Using both in vivo and ex vivo imaging, we identified a retinal region with a primate fovea-like cone photoreceptor density but without the excavation of the inner retina. Similar anatomical structure observed in rare human subjects has been named fovea-plana. In addition, dogs with mutations in two different genes, that cause macular degeneration in humans, developed earliest disease at the newly-identified canine fovea-like area. Our results challenge the dogma that within the phylogenetic tree of mammals, haplorhine primates with a fovea are the sole lineage in which the retina has a central bouquet of cones. Furthermore, a predilection for naturally-occurring retinal degenerations to alter this cone-enriched area fills the void for a clinically-relevant animal model of human macular degenerations.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3944008 | PMC |
http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0090390 | PLOS |
Invest Ophthalmol Vis Sci
October 2024
Division of Experimental Retinal Therapies, Department of Clinical Sciences & Advanced Medicine, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.
Vet Ophthalmol
September 2024
Department of Clinical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, New York, USA.
Objective: To describe the in vivo confocal microscopy (IVCM) findings in dogs with primary corneal squamous cell carcinoma (SCC).
Animals: Eight dogs with primary corneal SCC.
Procedures: Dogs diagnosed with primary corneal SCC by histopathology were examined with a modified Heidelberg Retina tomograph and Rostock Cornea Module prior to surgical intervention.
Cell Death Dis
September 2024
Division of Experimental Retinal Therapies, Department of Clinical Sciences & Advanced Medicine, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, PA, USA.
PRLΔE1, a retina-specific isoform of prolactin, is expressed in multiple and diverse forms of canine inherited retinal degeneration (IRD). We find that while PRLΔE1 expression in rods is not associated with the initial phase of disease characterized by acute photoreceptor cell death, it is associated with the protracted phase of slow cell loss. Restoration of photoreceptors to a healthy state by gene-specific replacement therapy of individual IRDs successfully suppresses PRLΔE1 expression.
View Article and Find Full Text PDFFront Ophthalmol (Lausanne)
August 2024
Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, United States.
Aim: Retinal cell therapy modalities, in the category of advanced therapy medicinal products (ATMPs), are being developed to target several retinal diseases. Testing in large animal models (LAMs) is a crucial step in translating retinal ATMPs into clinical practice. However, challenges including budgetary and infrastructure constraints can hinder LAM research design and execution.
View Article and Find Full Text PDFClin Genet
January 2025
Christian-Albrechts-University Kiel, Institute of Biochemistry, Kiel, Germany.
Usher syndrome (USH) is the most common cause of deafblindness. USH is autosomal recessively inherited and characterized by rod-cone dystrophy or retinitis pigmentosa (RP), often accompanied by sensorineural hearing loss. Variants in >15 genes have been identified as causative for clinically and genetically distinct subtypes.
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