Kai Kang1, Minzhong Yu1,2. 1. a Department of Ophthalmic Research , Cole Eye Institute, Cleveland Clinic Foundation , Cleveland , OH 44195 , USA. 2. b Department of Ophthalmology , Cleveland Clinic Lerner College of Medicine of Case Western Reserve University , Cleveland , OH 44195 , USA.
Abstract
PURPOSE: Retinitis pigmentosa (RP) is a group of inherited diseases characterized by the death of rod photoreceptors, followed by the death of cone photoreceptors, progressively leading to partial or complete blindness. Currently no specific treatment is available for RP patients. Sulforaphane (SFN) has been confirmed to be an effective antioxidant in the treatment of many diseases. In this study, we tested the therapeutic effects of SFN against photoreceptor degeneration in Pde6brd10 mice. METHODS: rd10 mice and C57/BL6 wild-type (WT) mice were treated with SFN and saline, respectively, from P6 to P20. Electroretinography (ERG), terminal deoxynucleotidyl transferase dUTP nick end labeling and western blot were tested, respectively, at P21 for the analysis of retinal function, retinal cell apoptosis or death and the protein express of GRP78/BiP (TUNEL) as a marker of endoplasmic reticulum (ER) stress. RESULTS: Compared with the saline group, the SFN-treated group showed significantly higher ERG a-wave and b-wave amplitudes, less photoreceptor death, and the downregulation of GRP78/BiP. CONCLUSIONS: Our data showed that SFN ameliorated the retinal degeneration of rd10 mice, which is possibly related to the downregulation of GRP78 expression.
PURPOSE:Retinitis pigmentosa (RP) is a group of inherited diseases characterized by the death of rod photoreceptors, followed by the death of cone photoreceptors, progressively leading to partial or complete blindness. Currently no specific treatment is available for RPpatients. Sulforaphane (SFN) has been confirmed to be an effective antioxidant in the treatment of many diseases. In this study, we tested the therapeutic effects of SFN against photoreceptor degeneration in Pde6brd10mice. METHODS:rd10mice and C57/BL6 wild-type (WT) mice were treated with SFN and saline, respectively, from P6 to P20. Electroretinography (ERG), terminal deoxynucleotidyl transferase dUTP nick end labeling and western blot were tested, respectively, at P21 for the analysis of retinal function, retinal cell apoptosis or death and the protein express of GRP78/BiP (TUNEL) as a marker of endoplasmic reticulum (ER) stress. RESULTS: Compared with the saline group, the SFN-treated group showed significantly higher ERG a-wave and b-wave amplitudes, less photoreceptor death, and the downregulation of GRP78/BiP. CONCLUSIONS: Our data showed that SFN ameliorated the retinal degeneration of rd10mice, which is possibly related to the downregulation of GRP78 expression.
Authors: John D Clarke; Anna Hsu; David E Williams; Roderick H Dashwood; Jan F Stevens; Masayuki Yamamoto; Emily Ho Journal: Pharm Res Date: 2011-06-17 Impact factor: 4.200
Authors: B Chang; N L Hawes; M T Pardue; A M German; R E Hurd; M T Davisson; S Nusinowitz; K Rengarajan; A P Boyd; S S Sidney; M J Phillips; R E Stewart; R Chaudhury; J M Nickerson; J R Heckenlively; J H Boatright Journal: Vision Res Date: 2007-01-30 Impact factor: 1.886
Authors: Hong Pan; Meihua He; Ruixing Liu; Nicholas C Brecha; Albert Cheung Hoi Yu; Mingliang Pu Journal: PLoS One Date: 2014-12-03 Impact factor: 3.240
Authors: Paul Yang; Rachel Lockard; Hope Titus; Jordan Hiblar; Kyle Weller; Dahlia Wafai; Richard G Weleber; Robert M Duvoisin; Catherine W Morgans; Mark E Pennesi Journal: Invest Ophthalmol Vis Sci Date: 2020-08-03 Impact factor: 4.799