title: Rescue of photoreceptor degeneration by curcumin in transgenic rats with P23H rhodopsin mutation.
abstract: The P23H mutation in the rhodopsin gene causes rhodopsin misfolding, altered trafficking and formation of insoluble aggregates leading to photoreceptor degeneration and autosomal dominant retinitis pigmentosa (RP). There are no effective therapies to treat this condition. Compounds that enhance dissociation of protein aggregates may be of value in developing new treatments for such diseases. Anti-protein aggregating activity of curcumin has been reported earlier. In this study we present that treatment of COS-7 cells expressing mutant rhodopsin with curcumin results in dissociation of mutant protein aggregates and decreases endoplasmic reticulum stress. Furthermore we demonstrate that administration of curcumin to P23H-rhodopsin transgenic rats improves retinal morphology, physiology, gene expression and localization of rhodopsin. Our findings indicate that supplementation of curcumin improves retinal structure and function in P23H-rhodopsin transgenic rats. This data also suggest that curcumin may serve as a potential therapeutic agent in treating RP due to the P23H rhodopsin mutation and perhaps other degenerative diseases caused by protein trafficking defects.
authors: Jacobs Retina Center, Department of Ophthalmology, University of California San Diego, La Jolla, California, United States of America.
authors: Vidyullatha
authors: V
authors: Vasireddy
authors: Venkata R M
authors: VR
authors: Chavali
authors: Victory T
authors: VT
authors: Joseph
authors: Rajendra
authors: R
authors: Kadam
authors: Jonathan H
authors: JH
authors: Lin
authors: Jeffrey A
authors: JA
authors: Jamison
authors: Uday B
authors: UB
authors: Kompella
authors: Geereddy Bhanuprakash
authors: GB
authors: Reddy
authors: Radha
authors: R
authors: Ayyagari
mesh_terms: D000818:Animals; D019556:COS Cells; D002522:Chlorocebus aethiops; D003474:Curcumin / Q000494:pharmacology* / Q000627:therapeutic use*; D007150:Immunohistochemistry; D016297:Mutagenesis, Site-Directed; D016133:Polymerase Chain Reaction; D051381:Rats; D017207:Rats, Sprague-Dawley; D055647:Rats, Transgenic; D012160:Retina / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D012162:Retinal Degeneration / Q000188:drug therapy* / Q000378:metabolism*; D012243:Rhodopsin / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*
chemical_list: D012243:Rhodopsin; D003474:Curcumin