萝卜硫素对色素性视网膜炎Pde6小鼠模型视网膜变性的保护作用。
Protective effect of sulforaphane against retinal degeneration in the Pde6 mouse model of retinitis pigmentosa.
作者信息
Kang Kai, Yu Minzhong
机构信息
a Department of Ophthalmic Research , Cole Eye Institute, Cleveland Clinic Foundation , Cleveland , OH 44195 , USA.
b Department of Ophthalmology , Cleveland Clinic Lerner College of Medicine of Case Western Reserve University , Cleveland , OH 44195 , USA.
出版信息
Curr Eye Res. 2017 Dec;42(12):1684-1688. doi: 10.1080/02713683.2017.1358371. Epub 2017 Sep 22.
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 Pde6b 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.
目的
视网膜色素变性（RP）是一组遗传性疾病，其特征是视杆光感受器死亡，随后视锥光感受器死亡，逐渐导致部分或完全失明。目前尚无针对RP患者的特异性治疗方法。萝卜硫素（SFN）已被证实是治疗多种疾病的有效抗氧化剂。在本研究中，我们测试了SFN对Pde6b小鼠光感受器变性的治疗效果。
方法
从出生后第6天（P6）至第20天（P20），分别用SFN和生理盐水处理rd10小鼠和C57/BL6野生型（WT）小鼠。在P21分别进行视网膜电图（ERG）、末端脱氧核苷酸转移酶介导的dUTP缺口末端标记法（TUNEL）和蛋白质免疫印迹法检测，以分析视网膜功能、视网膜细胞凋亡或死亡情况以及作为内质网（ER）应激标志物的葡萄糖调节蛋白78（GRP78）/免疫球蛋白重链结合蛋白（BiP）的蛋白质表达。
结果
与生理盐水组相比，SFN处理组的ERG a波和b波振幅显著更高，光感受器死亡更少，且GRP78/BiP表达下调。
结论
我们的数据表明，SFN改善了rd10小鼠的视网膜变性，这可能与GRP78表达下调有关。
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