Increased cone sensitivity to ABCA4 deficiency provides insight into macular vision loss in Stargardt's dystrophy

► Stargardt's disease is associated with mutations in Abca4 and causes macular degeneration. ► We use the Abca4−/−/Nrl−/− double knockout mouse to model Stargardt's disease. ► We show that cones accumulate more toxic A2E per mole of retinoid than rods. ► We also show that cones lacking Abca4 are less effective at clearing A2E than rods. ► These data suggest that cones are more sensitive to the loss of Abca4 than rods.