PDI在脊髓侧索硬化症中的研究进展  

Research progress of PDI in amyotrophic lateral sclerosis

作　　者：张永雪 蒲泽瑶 安亚婷 张薇[1] 王艺颖 郝瀚[1] 张会然[1] 张海林[1] 杜肖娜[1] ZHANG Yong-xue;PU Ze-yao;AN Ya-ting;ZHANG Wei;WANG Yi-ying;HAO Han;ZHANG Hui-ran;ZHANG Hai-lin;DU Xiao-na(Department of Pharmacology,Hebei Medical University,Shijiazhuang 050017,China;Department of Pharmacy,Handan First Hospital,Handan 056002,China)

机构地区：[1]河北医科大学药理学教研室,河北石家庄050017 [2]邯郸市第一医院药学部,河北邯郸056002

出　　处：《药学学报》2021年第10期2735-2741,共7页Acta Pharmaceutica Sinica

基　　金：国家自然科学基金资助项目(81870872);河北省教育厅在读研究生创新能力培养资助项目(CXZZBS2018074).

摘　　要：蛋白质二硫键异构酶(protein disulfide isomerase,PDI,又称PDIA1)是蛋白质二硫键异构酶家族的原型蛋白,主要存在于内质网中,可以通过酶活性和分子伴侣功能帮助蛋白正确折叠,参与生理或病理过程。脊髓侧索硬化症(amyotrophic lateral sclerosis,ALS)是一种神经退行性疾病,以蛋白异常聚集为主要特征。近年来有研究者发现ALS患者有PDI突变体,并证实PDI蛋白在ALS疾病发生发展过程中有重要作用。本文重点综述PDI蛋白在ALS研究中的进展,以期为ALS疾病的治疗提供新靶点。Protein disulfide isomerase(PDI),also named PDIA1,is an archetype member of protein disulfide isomerase family,which mainly exists in endoplasmic reticulum and plays an important role in pathophysiological progress via enzyme activity and molecular chaperone function helping protein folding correctly.Amyotrophic lateral sclerosis(ALS)is a kind of neurodegenerative diseases characterized by abnormal protein aggregation.Recently,PDI mutants have been found in ALS patients and the important role of PDI protein in the development of ALS has also been confirmed.Here,we focus on the recent studies of PDI in ALS hoping to provide a novel target for the treatment of ALS.

关 键 词：蛋白质二硫键异构酶 分子伴侣 脊髓侧索硬化症 神经退行性疾病 

分 类 号：R966[医药卫生—药理学]