title: Is There Excess Oxidative Stress and Damage in Eyes of Patients with Retinitis Pigmentosa?
abstract: Retinitis pigmentosa (RP) is a group of diseases in which a mutation in one of the large variety of genes causes death of rod photoreceptors. After rods die, cone photoreceptors gradually die resulting in constriction of visual fields and eventual blindness in many patients. Studies in animal models of RP have demonstrated that oxidative damage is a major contributor to cone cell death. In this study, we extended those findings to patients with RP, because compared to control patients, those with RP showed significant reduction in the reduced to oxidized glutathione (GSH/GSSG) ratio in aqueous humor and a significant increase in aqueous protein carbonyl content. In contrast, there was no significant decrease in the serum GSH/GSSG ratio or increase in carbonyl content of serum proteins. These data indicate that patients with RP have ocular oxidative stress and damage in the absence of manifestations of systemic oxidative stress and/or damage indicating that demonstrations of oxidative damage-induced cone cell death in animal models of RP may translate to human RP. These observations lead to the hypothesis that potent antioxidants will promote cone survival and function in patients with RP and that the aqueous GSH/GSSG ratio and carbonyl content on proteins may provide useful biomarkers. Antioxid. Redox Signal. 23, 643-648.
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Peter A
authors.initials: PA
authors.lastname: Campochiaro
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Rupert W
authors.initials: RW
authors.lastname: Strauss
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Lili
authors.initials: L
authors.lastname: Lu
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Gulnar
authors.initials: G
authors.lastname: Hafiz
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Yulia
authors.initials: Y
authors.lastname: Wolfson
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Syed M
authors.initials: SM
authors.lastname: Shah
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Raafay
authors.initials: R
authors.lastname: Sophie
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Tahreem A
authors.initials: TA
authors.lastname: Mir
authors.affiliation: Departments of Ophthalmology and Neuroscience, Johns Hopkins University School of Medicine , Baltimore, Maryland.
authors.forename: Hendrik P
authors.initials: HP
authors.lastname: Scholl
mesh_terms: D000818:Animals; D001082:Aqueous Humor / Q000378:metabolism*; D005123:Eye / Q000378:metabolism* / Q000473:pathology; D005978:Glutathione / Q000097:blood / Q000378:metabolism; D019803:Glutathione Disulfide / Q000378:metabolism; D006801:Humans; D018384:Oxidative Stress; D050050:Protein Carbonylation; D012174:Retinitis Pigmentosa / Q000378:metabolism / Q000473:pathology*
chemical_list: D005978:Glutathione; D019803:Glutathione Disulfide
references.citation: Invest Ophthalmol Vis Sci. 2000 Nov;41(12):3999-4006
references.pmid: 11053305
references.citation: Methods Enzymol. 1990;186:407-21
references.pmid: 2233308
references.citation: J Cell Physiol. 2005 Jun;203(3):457-64
references.pmid: 15744744
references.citation: Proc Natl Acad Sci U S A. 2006 Jul 25;103(30):11300-5
references.pmid: 16849425
references.citation: Mol Ther. 2009 May;17(5):778-86
references.pmid: 19293779
references.citation: J Neurochem. 2009 Aug;110(3):1028-37
references.pmid: 19493169
references.citation: J Cell Physiol. 2011 Jul;226(7):1843-9
references.pmid: 21506115
references.citation: Free Radic Biol Med. 2011 Oct 1;51(7):1347-54
references.pmid: 21736939
references.citation: PLoS One. 2013;8(9):e74223
references.pmid: 24069283