内质网应激在视网膜色素变性中的作用

Role of endoplasmic reticulum stress in retinitis pigmentosa

视网膜色素变性(RP)是以光感受器及色素上皮功能丧失为特征的退行性致盲疾病。内质网应激的激活是细胞的防御调节机制,旨在通过一系列分子信号通路进行自我调节以恢复内质网功能的稳定。视紫红质突变是RP的常见病因,内质网内视紫红质错误折叠和滞留,内质网应激诱发感光细胞和视网膜色素上皮细胞凋亡,均可导致RP的发生和发展。文章论述了内质网应激与其在RP发病机制中的作用,并对内质网应激抑制剂、中药和化学药物调控内质网应激在RP治疗中的作用进行总结,以期为内质网应激在RP的临床应用中提供理论依据,为RP的研究、预防和治疗提供新的思路。

Retinitis pigmentosa(RP)is a degenerative blinding disease characterized by the loss of the function of photoreceptor and retinal pigment epithelium.The activation of endoplasmic reticulum stress is a cellular defense regulatory mechanism,aimed at restoring the stability of endoplasmic reticulum function by self-regulation through a series of molecular signaling pathways.Rhodopsin mutation is a common cause of RP.Misfolding and retention of rhodopsin in endoplasmic reticulum and apoptosis of photoreceptor cells and retinal pigment epithelial cells induced by endoplasmic reticulum stress can lead to the occurrence and development of RP.This paper discusses endoplasmic reticulum stress and its role in the pathogenesis of RP and the role of endoplasmic reticulum stress inhibitors,traditional Chinese medicine and chemical drugs in regulating endoplasmic reticulum stress in RP treatment was summarized,in order to provide theoretical basis for endoplasmic reticulum stress in the clinical application of RP and provide new ideas for the research,prevention and treatment of RP.