Targeting the Proteostasis Network in Rhodopsin Retinitis Pigmentosa

Adv Exp Med Biol. 2016:854:479-84. doi: 10.1007/978-3-319-17121-0_64.

Abstract

Mutations in rhodopsin are one of the most common causes of retinitis pigmentosa (RP). Misfolding of rhodopsin can result in disruptions in cellular protein homeostasis, or proteostasis. There is currently no available treatment for RP. In this review, we discuss the different approaches currently being investigated for treatment of rhodopsin RP, focusing on the potential of manipulation of the proteostasis network as a therapeutic approach to combat retinal degeneration.

Keywords: ERAD; Heat shock proteins; Molecular chaperones; P23H; Proteostasis; Retinal degeneration; Retinitis pigmentosa; Rhodopsin.

Publication types

  • Review

MeSH terms

  • Animals
  • Disease Models, Animal
  • Genetic Predisposition to Disease / genetics*
  • Humans
  • Molecular Targeted Therapy / methods
  • Mutation*
  • Proteostasis Deficiencies / drug therapy
  • Proteostasis Deficiencies / genetics*
  • Retinaldehyde / therapeutic use
  • Retinitis Pigmentosa / drug therapy
  • Retinitis Pigmentosa / genetics*
  • Rhodopsin / chemistry
  • Rhodopsin / genetics*

Substances

  • Rhodopsin
  • Retinaldehyde