Bisretinoids as a Source of Early Photoreceptor Pathology in Stargardt Disease

Early recognition and treatment of patients with STGD1 who have relatively healthy retinal tissue will likely yield a more favorable visual prognosis. Accordingly, there is a need to identify early disease initiators and progression patterns. The reviewed data support the hypothesis that bisretinoid accumulation within photoreceptors may be responsible for the observed early retinal pathology and vision loss. Clinical evaluation of therapeutics intended to reduce bisretinoid accumulation in early-stage STGD1 patients will likely provide a greater understanding of the role of bisretinoids in disease progression and potential for vision preservation.