Figure 1.

Schematic steps in progressive retinal degeneration. Mutant-proteins accumulate during retinal degeneration and can be seen as new, which can amplify the autoimmune response, ultimately leading to autoimmune pathology. Activation of immune cells by overproduction of cellular debris due to photoreceptor death results in the inner Blood Retina Barrier (BRB) breakdown, which invites systemic macrophages into the retina. Resident and circulating macrophages can contribute to secondary retinal damage from inflammation, and in effect ameliorate retinal degeneration.