ER stress protects from retinal degeneration

EMBO J. 2009 May 6;28(9):1296-307. doi: 10.1038/emboj.2009.76. Epub 2009 Apr 2.

Abstract

The unfolded protein response (UPR) is a specific cellular process that allows the cell to cope with the overload of unfolded/misfolded proteins in the endoplasmic reticulum (ER). ER stress is commonly associated with degenerative pathologies, but its role in disease progression is still a matter for debate. Here, we found that mutations in the ER-resident chaperone, neither inactivation nor afterpotential A (NinaA), lead to mild ER stress, protecting photoreceptor neurons from various death stimuli in adult Drosophila. In addition, Drosophila S2 cultured cells, when pre-exposed to mild ER stress, are protected from H(2)O(2), cycloheximide- or ultraviolet-induced cell death. We show that a specific ER-mediated signal promotes antioxidant defences and inhibits caspase-dependent cell death. We propose that an immediate consequence of the UPR not only limits the accumulation of misfolded proteins but also protects tissues from harmful exogenous stresses.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis / drug effects
  • Caspases / metabolism
  • Cell Line
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / physiology
  • Drosophila Proteins / genetics
  • Drosophila Proteins / physiology
  • Drosophila melanogaster / cytology
  • Drosophila melanogaster / drug effects
  • Drosophila melanogaster / physiology*
  • Endoplasmic Reticulum / metabolism
  • Endoplasmic Reticulum / physiology*
  • Hydrogen Peroxide / pharmacology
  • Membrane Proteins / genetics
  • Membrane Proteins / physiology
  • Molecular Chaperones / genetics
  • Molecular Chaperones / physiology
  • Mutation
  • Photoreceptor Cells / cytology
  • Photoreceptor Cells / drug effects
  • Photoreceptor Cells / metabolism
  • Retina / cytology
  • Retina / drug effects
  • Retina / metabolism
  • Retinal Degeneration / genetics
  • Retinal Degeneration / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Stress, Physiological / genetics
  • Stress, Physiological / physiology*

Substances

  • DNA-Binding Proteins
  • Drosophila Proteins
  • Membrane Proteins
  • Molecular Chaperones
  • Xbp1 protein, Drosophila
  • ninaA protein, Drosophila
  • Hydrogen Peroxide
  • Caspases