The cell stress machinery and retinal degeneration

FEBS Lett. 2013 Jun 27;587(13):2008-17. doi: 10.1016/j.febslet.2013.05.020. Epub 2013 May 15.


Retinal degenerations are a group of clinically and genetically heterogeneous disorders characterised by progressive loss of vision due to neurodegeneration. The retina is a highly specialised tissue with a unique architecture and maintaining homeostasis in all the different retinal cell types is crucial for healthy vision. The retina can be exposed to a variety of environmental insults and stress, including light-induced damage, oxidative stress and inherited mutations that can lead to protein misfolding. Within retinal cells there are different mechanisms to cope with disturbances in proteostasis, such as the heat shock response, the unfolded protein response and autophagy. In this review, we discuss the multiple responses of the retina to different types of stress involved in retinal degenerations, such as retinitis pigmentosa, age-related macular degeneration and glaucoma. Understanding the mechanisms that maintain and re-establish proteostasis in the retina is important for developing new therapeutic approaches to fight blindness.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • Mutation
  • Proteostasis Deficiencies / metabolism
  • Proteostasis Deficiencies / pathology
  • Proteostasis Deficiencies / therapy
  • Retina / metabolism
  • Retina / pathology
  • Retinal Degeneration / metabolism*
  • Retinal Degeneration / pathology
  • Retinal Degeneration / therapy
  • Retinal Pigment Epithelium / metabolism*
  • Retinal Pigment Epithelium / pathology
  • Retinal Vessels / pathology
  • Rhodopsin / genetics
  • Rhodopsin / metabolism
  • Stress, Physiological*


  • Rhodopsin