Nucleo-cytoplasmic transport defects and protein aggregates in neurodegeneration

• 作者: Giacomo Bitetto Alessio Di Fonzo
• 来源期刊: 转化神经变性病(英文)
• 学科: 医学
• 关键词: AGING NEURODEGENERATION Neurodegenerative disease Nucleo-cytoplasmic transport Nuclear pore complex Protein aggregate
• 年，卷（期）: zhsjbxbyw_2020,（3）
• 页码范围: 354-369
• 页数: 16页
• 分类号: R73

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.