Chemiexcitation and melanin in photoreceptor disc turnover and prevention of macular degeneration.

ABSTRACT: Age-related macular degeneration, Stargardt disease, and their Abca4^-/- mouse model are characterized by accelerated accumulation of the pigment lipofuscin, derived from photoreceptor disc turnover in the retinal pigment epithelium (RPE); lipofuscin accumulation and retinal degeneration both occur earlier in albino mice. Intravitreal injection of superoxide (O₂^•-) generators reverses lipofuscin accumulation and rescues retinal pathology, but neither the target nor mechanism is known. Here we show that RPE contains thin multi-lamellar membranes (TLMs) resembling photoreceptor discs, which associate with melanolipofuscin granules in pigmented mice but in albinos are 10-fold more abundant and reside in vacuoles. Genetically over-expressing tyrosinase in

SUBMITTER: Lyu Y 

PROVIDER: S-EPMC10194005 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature