Increased proteasomal activity supports photoreceptor survival in inherited retinal degeneration

Abstract

Proteasomal overload can be found in a broad spectrum of mouse models of retinal degeneration. Here the authors find that overexpressing the PA28α subunit of the 11S proteasome cap increased the number of surviving functional photoreceptor cells in a mouse model of retinal degeneration bearing the P23H mutation in rhodopsin.