• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

原纤维是否为帕金森病的模型?

Are Preformed Fibrils a Model of Parkinson's Disease?

机构信息

Department of Microbiology, Immunology, and Pathology, Prion Research Center, Colorado State University, Fort Collins, CO, USA.

Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, University of Pennsylvania, Philadelphia, PA, USA.

出版信息

J Parkinsons Dis. 2024;14(6):1095-1103. doi: 10.3233/JPD-240228.

DOI:10.3233/JPD-240228
PMID:39031387
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11380230/
Abstract

Pre-formed fibrils (PFFs) made from recombinant α-synuclein are broadly used throughout the field in cellular and animal models of Parkinson's disease. However, their ability to successfully recapitulate disease biology is a controversial topic. In this article, two researchers debate this issue with Amanda Woerman taking the view that PFFs are a model of synucleinopathy but not Parkinson's disease, while Kelvin Luk defends their use as an important tool in the field.

摘要

由重组α-突触核蛋白制成的预形成纤维(PFFs)在帕金森病的细胞和动物模型领域被广泛应用。然而,它们成功再现疾病生物学的能力是一个有争议的话题。在这篇文章中,两位研究人员就此问题展开了辩论,Amanda Woerman 认为 PFFs 是一种突触核蛋白病模型,但不是帕金森病模型,而 Kelvin Luk 则为其作为该领域的重要工具进行了辩护。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/2be6cc3cba8d/jpd-14-jpd240228-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/d730a521cc86/jpd-14-jpd240228-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/568209bbb4fc/jpd-14-jpd240228-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/2be6cc3cba8d/jpd-14-jpd240228-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/d730a521cc86/jpd-14-jpd240228-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/568209bbb4fc/jpd-14-jpd240228-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebfa/11380230/2be6cc3cba8d/jpd-14-jpd240228-g003.jpg

相似文献

1
Are Preformed Fibrils a Model of Parkinson's Disease?原纤维是否为帕金森病的模型?
J Parkinsons Dis. 2024;14(6):1095-1103. doi: 10.3233/JPD-240228.
2
Brain-derived and in vitro-seeded alpha-synuclein fibrils exhibit distinct biophysical profiles.脑源性和体外接种的α-突触核蛋白纤维表现出不同的生物物理特征。
Elife. 2024 Nov 25;13:RP92775. doi: 10.7554/eLife.92775.
3
Transmission of peripheral blood α-synuclein fibrils exacerbates synucleinopathy and neurodegeneration in Parkinson's disease by endothelial Lag3 endocytosis.外周血α-突触核蛋白原纤维的传播通过内皮细胞Lag3内吞作用加剧帕金森病中的突触核蛋白病和神经退行性变。
Am J Physiol Cell Physiol. 2025 Mar 1;328(3):C836-C855. doi: 10.1152/ajpcell.00639.2024. Epub 2024 Dec 9.
4
Analysis of hemisphere-dependent effects of unilateral intrastriatal injection of α-synuclein pre-formed fibrils on mitochondrial protein levels, dynamics, and function.分析单侧纹状体注射α-突触核蛋白原纤维对线粒体蛋白水平、动态和功能的半球依赖性影响。
Acta Neuropathol Commun. 2022 May 23;10(1):78. doi: 10.1186/s40478-022-01374-z.
5
Current safety recommendations for handling mouse and human αsynuclein pre-formed fibrils.处理小鼠和人类α-突触核蛋白预形成纤维的当前安全建议。
Neurobiol Dis. 2025 Mar;206:106820. doi: 10.1016/j.nbd.2025.106820. Epub 2025 Jan 30.
6
Sequential or Simultaneous Injection of Preformed Fibrils and AAV Overexpression of Alpha-Synuclein Are Equipotent in Producing Relevant Pathology and Behavioral Deficits.预先形成的原纤维的序贯或同时注射以及α-突触核蛋白的腺相关病毒过表达在产生相关病理和行为缺陷方面具有同等效力。
J Parkinsons Dis. 2022;12(4):1133-1153. doi: 10.3233/JPD-212555.
7
Intracranial administration of alpha-synuclein fibrils in A30P-synuclein transgenic mice causes robust synucleinopathy and microglial induction.在 A30P-突触核蛋白转基因小鼠中脑内给予α-突触核蛋白纤维导致强烈的突触核蛋白病和小胶质细胞诱导。
Neurobiol Aging. 2021 Oct;106:12-25. doi: 10.1016/j.neurobiolaging.2021.05.012. Epub 2021 Jul 3.
8
Synthetic alpha-synuclein fibrils cause mitochondrial impairment and selective dopamine neurodegeneration in part via iNOS-mediated nitric oxide production.合成的α-突触核蛋白原纤维部分通过诱导型一氧化氮合酶介导的一氧化氮生成导致线粒体损伤和选择性多巴胺能神经变性。
Cell Mol Life Sci. 2017 Aug;74(15):2851-2874. doi: 10.1007/s00018-017-2541-x. Epub 2017 May 22.
9
Trans-synaptic and retrograde axonal spread of Lewy pathology following pre-formed fibril injection in an in vivo A53T alpha-synuclein mouse model of synucleinopathy.在活体 A53T α-突触核蛋白帕金森病小鼠模型中,预形成纤维注射后路易体病理学的突触前和逆行轴突播散。
Acta Neuropathol Commun. 2020 Aug 28;8(1):150. doi: 10.1186/s40478-020-01026-0.
10
Preformed fibrils generated from mouse alpha-synuclein produce more inclusion pathology in rats than fibrils generated from rat alpha-synuclein.由鼠源 α-突触核蛋白生成的预制纤维比由大鼠源 α-突触核蛋白生成的纤维在大鼠中产生更多的包涵体病理学。
Parkinsonism Relat Disord. 2021 Aug;89:41-47. doi: 10.1016/j.parkreldis.2021.06.010. Epub 2021 Jun 19.

引用本文的文献

1
Co-infection with two α-synuclein strains reveals novel synergistic interactions.两种α-突触核蛋白毒株的共同感染揭示了新的协同相互作用。
bioRxiv. 2025 Aug 22:2025.08.17.670736. doi: 10.1101/2025.08.17.670736.
2
A-synuclein prion strains differentially adapt after passage in mice.α-突触核蛋白朊病毒株在小鼠体内传代后会出现不同的适应性变化。
PLoS Pathog. 2024 Dec 6;20(12):e1012746. doi: 10.1371/journal.ppat.1012746. eCollection 2024 Dec.

本文引用的文献

1
Structure of alpha-synuclein fibrils derived from human Lewy body dementia tissue.来源于人类路易体痴呆组织的α-突触核蛋白纤维的结构。
Nat Commun. 2024 Mar 29;15(1):2750. doi: 10.1038/s41467-024-46832-5.
2
Spatial transcriptomics reveals molecular dysfunction associated with cortical Lewy pathology.空间转录组学揭示了与皮质路易体病理相关的分子功能障碍。
Nat Commun. 2024 Mar 26;15(1):2642. doi: 10.1038/s41467-024-47027-8.
3
A biological classification of Parkinson's disease: the SynNeurGe research diagnostic criteria.帕金森病的生物学分类:SynNeurGe 研究诊断标准。
Lancet Neurol. 2024 Feb;23(2):191-204. doi: 10.1016/S1474-4422(23)00404-0.
4
α-Synuclein aggregates amplified from patient-derived Lewy bodies recapitulate Lewy body diseases in mice.从患者来源的路易小体中扩增的α-突触核蛋白聚集物在小鼠中再现了路易体疾病。
Nat Commun. 2023 Oct 28;14(1):6892. doi: 10.1038/s41467-023-42705-5.
5
Hippocampal subfield vulnerability to α-synuclein pathology precedes neurodegeneration and cognitive dysfunction.海马亚区对α-突触核蛋白病变的易损性先于神经退行性变和认知功能障碍。
NPJ Parkinsons Dis. 2023 Aug 29;9(1):125. doi: 10.1038/s41531-023-00574-1.
6
α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies.α-突触核蛋白构象揭示了与 α-突触核蛋白病临床异质性的联系。
Transl Neurodegener. 2023 Mar 14;12(1):12. doi: 10.1186/s40035-023-00342-4.
7
New SNCA mutation and structures of α-synuclein filaments from juvenile-onset synucleinopathy.新型 SNCA 突变与早发性突触核蛋白病中α-突触核蛋白丝的结构
Acta Neuropathol. 2023 May;145(5):561-572. doi: 10.1007/s00401-023-02550-8. Epub 2023 Feb 27.
8
Cryo-EM of prion strains from the same genotype of host identifies conformational determinants.同种宿主来源的朊病毒株的低温电子显微镜分析确定构象决定因素。
PLoS Pathog. 2022 Nov 7;18(11):e1010947. doi: 10.1371/journal.ppat.1010947. eCollection 2022 Nov.
9
Quaternary structure of patient-homogenate amplified α-synuclein fibrils modulates seeding of endogenous α-synuclein.患者匀浆扩增的α-突触核蛋白纤维的四聚体结构调节内源性α-突触核蛋白的种子形成。
Commun Biol. 2022 Sep 30;5(1):1040. doi: 10.1038/s42003-022-03948-y.
10
Structures of α-synuclein filaments from human brains with Lewy pathology.具有路易体病理的人脑α-突触核蛋白纤维的结构。
Nature. 2022 Oct;610(7933):791-795. doi: 10.1038/s41586-022-05319-3. Epub 2022 Sep 15.