未折叠蛋白反应在角膜营养不良中的研究进展  

Research Progress of Unfolded Protein Response in Corneal Dystrophy

作　　者：刘学睿 王丽媛[1] 张毅[1] 郑涛 赵楚楚[1] 刘平[1] LIU Xuerui;WANG Liyuan;ZHANG Yi;ZHENG Tao;ZHAO Chuchu;LIU Ping(Department of Ophthalmology,the First Affiliated Hospital of Harbin Medical University,Harbin 150001,China)

机构地区：[1]哈尔滨医科大学附属第一医院眼科,哈尔滨150001

出　　处：《医学综述》2021年第12期2327-2332,共6页Medical Recapitulate

基　　金：黑龙江省博士后基金(LBH-Z18185)。

摘　　要：角膜营养不良是一种双侧进展性的、非炎性遗传性疾病。晚期角膜营养不良常导致视力明显下降,目前非角膜移植无法治愈,且为全球角膜移植常见的原因之一。角膜营养不良的发病机制目前尚不清楚。未折叠蛋白反应是一个复杂的信号通路,其维持着细胞内质网腔中蛋白质的动态平衡,与角膜营养不良相关。在角膜营养不良中,未折叠蛋白反应过度激活,无法维持蛋白质稳态,并可能通过激活凋亡信号通路促进细胞死亡。未来未折叠蛋白反应可能作为角膜营养不良的治疗靶点。Corneal dystrophies are broadly defined as inherited disorders that are usually progressive,bilateral and non-inflammatory conditions.The vision of patients with advanced corneal dystrophy is often significantly decreased.At present,only corneal transplantation can save the vision,and corneal dystrophy is one of the common causes of corneal transplantation worldwide.The pathogenesis of corneal dystrophy is still unclear.The unfolded protein response is a complex signaling pathway and maintains the dynamic balance of proteins in the endoplasmic reticulum cavity,which is related to corneal dystrophy.In corneal dystrophy,the unfolded protein response is overactivated,which cannot maintain protein homeostasis,and may promote cell death by activating the apoptotic signaling pathway.In the future,the unfolded protein response can be used as a target for the treatment of corneal dystrophy.

关 键 词：角膜营养不良 未折叠蛋白反应 凋亡 

分 类 号：R772.2[医药卫生—眼科]