Intraretinal Variation in Disease Severity in the Oat Mouse Model of Gyrate Atrophy

The study on the Oatrhg mouse model of gyrate atrophy reveals that despite a 15-fold increase in plasma ornithine levels, these mice show minimal deficits in overall retinal structure and function compared to wild type mice. However, using OCT and cSLO, researchers found distinct patches of atrophy in the superonasal retina in 13 out of 18 Oatrhg mice, with some also showing atrophy in the inferior retina. This suggests regional differences in disease manifestation, with significant RPE cell hypertrophy and disorganization in the superonasal region potentially preceding photoreceptor loss. The study underscores the importance of considering genetic background and intraretinal variability in future research and highlights the value of non-invasive imaging techniques for early detection and understanding of gyrate atrophy.