title: Targeting the Proteostasis Network in Rhodopsin Retinitis Pigmentosa.
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.
authors.affiliation: Ocular Biology and Therapeutics, UCL Institute of Ophthalmology, 11-43 Bath Street, EC1V 9EL, London, UK. d.parfitt@ucl.ac.uk.
authors.forename: David A
authors.initials: DA
authors.lastname: Parfitt
authors.affiliation: Ocular Biology and Therapeutics, UCL Institute of Ophthalmology, 11-43 Bath Street, EC1V 9EL, London, UK. michael.cheetham@ucl.ac.uk.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Cheetham
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D058990:Molecular Targeted Therapy / Q000379:methods; D009154:Mutation; D057165:Proteostasis Deficiencies / Q000188:drug therapy / Q000235:genetics*; D012172:Retinaldehyde / Q000627:therapeutic use; D012174:Retinitis Pigmentosa / Q000188:drug therapy / Q000235:genetics*; D012243:Rhodopsin / Q000737:chemistry / Q000235:genetics*
chemical_list: D012243:Rhodopsin; D012172:Retinaldehyde
keywords: ERAD; Heat shock proteins; Molecular chaperones; P23H; Proteostasis; Retinal degeneration; Retinitis pigmentosa; Rhodopsin