Nucleo-cytoplasmic transport defects and protein aggregates in neurodegeneration

作     者：Giacomo Bitetto Alessio Di Fonzo 

作者机构：IRCCS Foundation Ca'Granda Ospedale Maggiore PoliclinicoDino Ferrari CenterNeuroscience SectionDepartment of Pathophysiology and TransplantationUniversity of MilanVia Francesco Sforza 3520122 MilanItaly 

出 版 物：《Translational Neurodegeneration》 (转化神经变性病（英文）)

年 卷 期：2020年第9卷第3期

页      面：354-369页

核心收录：

学科分类：0710[理学-生物学] 0402[教育学-心理学(可授教育学、理学学位）] 1002[医学-临床医学] 1001[医学-基础医学(可授医学、理学学位)] 1010[医学-医学技术（可授医学、理学学位）] 10[医学] 1009[医学-特种医学] 

基　　金：Fresco Institute 

主　　题：Aging Neurodegeneration Neurodegenerative disease Nucleo-cytoplasmic transport Nuclear pore complex Protein aggregate 

摘      要：In the ongoing process of uncovering molecular abnormalities in neurodegenerative diseases characterized by toxic protein aggregates,nucleo-cytoplasmic transport defects have an emerging role.Several pieces of evidence suggest a link between neuronal protein inclusions and nuclear pore complex(NPC)damage.These processes lead to oxidative stress,inefficient transcription,and aberrant DNAVRNA maintenance.The clinical and neuropathological spectrum of NPC defects is broad,ranging from physiological aging to a suite of neurodegenerative diseases.A better understanding of the shared pathways among these conditions may represent a significant step toward dissecting their underlying molecular mechanisms,opening the way to a real possibility of identifying common therapeutic targets.