突触核蛋白病脑组织α-突触核蛋白细丝超微结构及实验模型。

IF 2.5 4区 医学 Q2 CLINICAL NEUROLOGY Journal of Movement Disorders Pub Date : 2024-01-01 Epub Date: 2023-11-22 DOI:10.14802/jmd.23213
Airi Tarutani, Masato Hasegawa
{"title":"突触核蛋白病脑组织α-突触核蛋白细丝超微结构及实验模型。","authors":"Airi Tarutani, Masato Hasegawa","doi":"10.14802/jmd.23213","DOIUrl":null,"url":null,"abstract":"<p><p>Intracellular α-synuclein (α-syn) inclusions are a neuropathological hallmark of Lewy body disease (LBD) and multiple system atrophy (MSA), both of which are termed synucleinopathies. LBD is defined by Lewy bodies and Lewy neurites in neurons, while MSA displays glial cytoplasmic inclusions in oligodendrocytes. Pathological α-syn adopts an ordered filamentous structure with a 5-10 nm filament diameter, and this conformational change has been suggested to be involved in the disease onset and progression. Synucleinopathies also exhibit characteristic ultrastructural and biochemical properties of α-syn filaments, and α-syn strains with distinct conformations have been identified. Numerous experimental studies have supported the idea that pathological α-syn self-amplifies and spreads throughout the brain, during which processes the conformation of α-syn filaments may drive the disease specificity. In this review, we summarize the ultrastructural features and heterogeneity of α-syn filaments in the brains of patients with synucleinopathy and in experimental models of seeded α-syn aggregation.</p>","PeriodicalId":16372,"journal":{"name":"Journal of Movement Disorders","volume":" ","pages":"15-29"},"PeriodicalIF":2.5000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10846975/pdf/","citationCount":"0","resultStr":"{\"title\":\"Ultrastructures of α-Synuclein Filaments in Synucleinopathy Brains and Experimental Models.\",\"authors\":\"Airi Tarutani, Masato Hasegawa\",\"doi\":\"10.14802/jmd.23213\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Intracellular α-synuclein (α-syn) inclusions are a neuropathological hallmark of Lewy body disease (LBD) and multiple system atrophy (MSA), both of which are termed synucleinopathies. LBD is defined by Lewy bodies and Lewy neurites in neurons, while MSA displays glial cytoplasmic inclusions in oligodendrocytes. Pathological α-syn adopts an ordered filamentous structure with a 5-10 nm filament diameter, and this conformational change has been suggested to be involved in the disease onset and progression. Synucleinopathies also exhibit characteristic ultrastructural and biochemical properties of α-syn filaments, and α-syn strains with distinct conformations have been identified. Numerous experimental studies have supported the idea that pathological α-syn self-amplifies and spreads throughout the brain, during which processes the conformation of α-syn filaments may drive the disease specificity. In this review, we summarize the ultrastructural features and heterogeneity of α-syn filaments in the brains of patients with synucleinopathy and in experimental models of seeded α-syn aggregation.</p>\",\"PeriodicalId\":16372,\"journal\":{\"name\":\"Journal of Movement Disorders\",\"volume\":\" \",\"pages\":\"15-29\"},\"PeriodicalIF\":2.5000,\"publicationDate\":\"2024-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10846975/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Movement Disorders\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.14802/jmd.23213\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2023/11/22 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q2\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Movement Disorders","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.14802/jmd.23213","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/11/22 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0

摘要

细胞内α-突触核蛋白(α-syn)包涵体是路易体病(LBD)和多系统萎缩(MSA)的神经病理学标志,这两种疾病都被称为突触核蛋白病。LBD由神经元中的路易小体和路易神经突定义,而MSA在少突胶质细胞中表现为胶质细胞质包涵体。病理性α-syn采用5-10 nm丝径的有序丝状结构,这种构象变化被认为参与了疾病的发生和发展。突触核蛋白病还表现出α-syn细丝特有的超微结构和生化特性,并且已经鉴定出具有不同构象的α-syn菌株。大量实验研究支持病理性α-syn自我放大并在整个大脑中扩散的观点,在此过程中α-syn细丝的构象过程可能驱动疾病特异性。本文综述了突触核蛋白病患者脑内α-syn细丝的超微结构特征和异质性,以及种子α-syn聚集的实验模型。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Ultrastructures of α-Synuclein Filaments in Synucleinopathy Brains and Experimental Models.

Intracellular α-synuclein (α-syn) inclusions are a neuropathological hallmark of Lewy body disease (LBD) and multiple system atrophy (MSA), both of which are termed synucleinopathies. LBD is defined by Lewy bodies and Lewy neurites in neurons, while MSA displays glial cytoplasmic inclusions in oligodendrocytes. Pathological α-syn adopts an ordered filamentous structure with a 5-10 nm filament diameter, and this conformational change has been suggested to be involved in the disease onset and progression. Synucleinopathies also exhibit characteristic ultrastructural and biochemical properties of α-syn filaments, and α-syn strains with distinct conformations have been identified. Numerous experimental studies have supported the idea that pathological α-syn self-amplifies and spreads throughout the brain, during which processes the conformation of α-syn filaments may drive the disease specificity. In this review, we summarize the ultrastructural features and heterogeneity of α-syn filaments in the brains of patients with synucleinopathy and in experimental models of seeded α-syn aggregation.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Journal of Movement Disorders
Journal of Movement Disorders CLINICAL NEUROLOGY-
CiteScore
2.50
自引率
5.10%
发文量
49
审稿时长
12 weeks
期刊最新文献
Brittle response to levodopa as a marker of Parkinson's disease phenotype characterized by heavy motor and non-motor burden. Clozapine-Induced Negative Myoclonus Leading to Recurrent Falls: A Case Report. Polysomnographic Findings into Sleep Disorders in Essential Tremor and Essential Tremor Plus: A Comparison with Healthy Controls. Taltirelin Hydrate in Patients with Ataxia Due to Spinocerebellar Degeneration. Myoclonic Epilepsy of Unverricht and Lundborg in a Filipino Woman.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1