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Abstract

Animal models are powerful tools to broaden our understanding in disease mechanisms and to develop future treatment strategies. Here we present detailed
structural and functional findings of a rhesus macaque suffering from a naturally occurring bilateral macular dystrophy (BMD), partial optic atrophy and corresponding
reduction of central V1 signals in visual fMRI experiments when compared to data in a healthy macaque (CTRL) of similar age. Fluorescence and indocyanine green
angiography showed reduced macular vascularization with significantly larger foveal avascular zones (FAZ) in the affected animal (FAZBMD = 8.85 mm2 vs. FAZCTRL =
0.32 mm2). Optical coherence tomography showed bilateral thinning of the macula within the FAZ (total retinal thickness, TRTBMD = 174 ± 9 μm) and partial optic nerve
atrophy when compared to control (TRTCTRL = 303 ± 45 μm). Segmentation analysis revealed that inner retinal layers were primarily affected (inner retinal thickness,
IRTBMD = 33 ± 9 μm vs. IRTCTRL = 143 ± 45 μm), while the outer retina essentially maintained its thickness (ORTBMD = 141 ± 7 μm vs. ORTCTRL = 160 ± 11 μm).
Accordingly, a strong central reduction in the multifocal electroretinography and a specific attenuation of cone-derived signals in Ganzfeld electroretinography was found, whereas rod function remained normal.
We provided detailed characterization of a primate macular disorder. This study aims to stimulate awareness and further investigation in primates with macular disorders
eventually leading to the identification of a primate animal model and facilitating the preclinical development of therapeutic strategies.