It is been long known that ventral and central cones degenerate more rapidly than dorsal cones in retinoid-deprived LCA (Leber’s Congenital Amaurosis) models. This paper in PNAS by Tao Zhang, Wolfgang Baehr…
…and Yingbin Fu shows that SW-opsins, but not ML-opsin, aggregates to form cytoplasmic inclusions in mutant mice and transfected cells. This result explains why blue cone function is lost earlier than red/green-cone function in patients with LCA. They also show that substituting rhodopsin with S-opsin in Lrat−/− rods resulted in mislocalization and aggregation of S-opsin, ER stress, and dramatically accelerated rod degeneration. According to these results, cone opsins may play a major role in determining the degeneration rate of photoreceptors in LCA.