A review of the mechanisms of cone degeneration in retinitis pigmentosa

DS Narayan, JPM Wood, G Chidlow… - Acta …, 2016 - Wiley Online Library
Acta ophthalmologica, 2016Wiley Online Library
Retinitis pigmentosa (RP) is an inherited condition that features degeneration of rod and
cone photoreceptors. In all forms of RP, the genetic mutation is expressed exclusively in
rods; however, cones die too. The secondary death of cones in RP remains somewhat
mysterious. A better understanding of the mechanisms that cause cone degeneration in RP
could lead to novel treatments that preserve cones. There are a number of prevailing
theories that attempt to explain cone degeneration in RP. One concept is that cone survival …
Abstract
Retinitis pigmentosa (RP) is an inherited condition that features degeneration of rod and cone photoreceptors. In all forms of RP, the genetic mutation is expressed exclusively in rods; however, cones die too. The secondary death of cones in RP remains somewhat mysterious. A better understanding of the mechanisms that cause cone degeneration in RP could lead to novel treatments that preserve cones. There are a number of prevailing theories that attempt to explain cone degeneration in RP. One concept is that cone survival is dependent on trophic factors produced by rods. Another hypothesis is that cones suffer from a nutrient shortage after rods have been lost. Additionally, oxidative stress and pro‐inflammatory microglial activation have also been suggested to play a role in cone death. The present review evaluates the evidence supporting these theories and provides an update on the mechanisms of cone degeneration in RP.
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