Peripheral proteinopathy in neurodegenerative diseases

Abstract Proteinopathies in neurology typically refer to pathological changes in proteins associated with neurological diseases, such as the aggregation of amyloid β and Tau in Alzheimer’s disease, α-synuclein in Parkinson’s disease and multiple system atrophy, and TAR DNA-binding protein 43 in amyo...

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Main Authors: Bin Xu, Xia Lei, Ying Yang, Jiayi Yu, Jun Chen, Zhi Xu, Keqiang Ye, Jing Zhang
Format: Article
Language:English
Published: BMC 2025-01-01
Series:Translational Neurodegeneration
Subjects:
Online Access:https://doi.org/10.1186/s40035-024-00461-6
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author Bin Xu
Xia Lei
Ying Yang
Jiayi Yu
Jun Chen
Zhi Xu
Keqiang Ye
Jing Zhang
author_facet Bin Xu
Xia Lei
Ying Yang
Jiayi Yu
Jun Chen
Zhi Xu
Keqiang Ye
Jing Zhang
author_sort Bin Xu
collection DOAJ
description Abstract Proteinopathies in neurology typically refer to pathological changes in proteins associated with neurological diseases, such as the aggregation of amyloid β and Tau in Alzheimer’s disease, α-synuclein in Parkinson’s disease and multiple system atrophy, and TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal dementia. Interestingly, these proteins are also commonly found in peripheral tissues, raising important questions about their roles in neurological disorders. Multiple studies have shown that peripherally derived pathological proteins not only travel to the brain through various routes, aggravating brain pathology, but also contribute significantly to peripheral dysfunction, highlighting their crucial impact on neurological diseases. Investigating how these peripherally derived proteins influence the progression of neurological disorders could open new horizons for achieving early diagnosis and treatment. This review summarizes the distribution, transportation pathways, and pathogenic mechanisms of several neurodegenerative disease-related pathological proteins in the periphery, proposing that targeting these peripheral pathological proteins could be a promising strategy for preventing and managing neurological diseases.
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institution Kabale University
issn 2047-9158
language English
publishDate 2025-01-01
publisher BMC
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series Translational Neurodegeneration
spelling doaj-art-c5065de84abb48e0bae1b3509e08b8822025-01-19T12:36:51ZengBMCTranslational Neurodegeneration2047-91582025-01-0114111410.1186/s40035-024-00461-6Peripheral proteinopathy in neurodegenerative diseasesBin Xu0Xia Lei1Ying Yang2Jiayi Yu3Jun Chen4Zhi Xu5Keqiang Ye6Jing Zhang7Department of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityDepartment of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityDepartment of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityDepartment of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityDepartment of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityDepartment of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityDepartment of Pathology and Laboratory Medicine, Emory University School of MedicineDepartment of Pathology, School of Medicine, The First Affiliated Hospital, Zhejiang UniversityAbstract Proteinopathies in neurology typically refer to pathological changes in proteins associated with neurological diseases, such as the aggregation of amyloid β and Tau in Alzheimer’s disease, α-synuclein in Parkinson’s disease and multiple system atrophy, and TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal dementia. Interestingly, these proteins are also commonly found in peripheral tissues, raising important questions about their roles in neurological disorders. Multiple studies have shown that peripherally derived pathological proteins not only travel to the brain through various routes, aggravating brain pathology, but also contribute significantly to peripheral dysfunction, highlighting their crucial impact on neurological diseases. Investigating how these peripherally derived proteins influence the progression of neurological disorders could open new horizons for achieving early diagnosis and treatment. This review summarizes the distribution, transportation pathways, and pathogenic mechanisms of several neurodegenerative disease-related pathological proteins in the periphery, proposing that targeting these peripheral pathological proteins could be a promising strategy for preventing and managing neurological diseases.https://doi.org/10.1186/s40035-024-00461-6Neurodegenerative diseasesPeripheral proteinopathiesAmyloid βTauα-SynucleinTDP-43
spellingShingle Bin Xu
Xia Lei
Ying Yang
Jiayi Yu
Jun Chen
Zhi Xu
Keqiang Ye
Jing Zhang
Peripheral proteinopathy in neurodegenerative diseases
Translational Neurodegeneration
Neurodegenerative diseases
Peripheral proteinopathies
Amyloid β
Tau
α-Synuclein
TDP-43
title Peripheral proteinopathy in neurodegenerative diseases
title_full Peripheral proteinopathy in neurodegenerative diseases
title_fullStr Peripheral proteinopathy in neurodegenerative diseases
title_full_unstemmed Peripheral proteinopathy in neurodegenerative diseases
title_short Peripheral proteinopathy in neurodegenerative diseases
title_sort peripheral proteinopathy in neurodegenerative diseases
topic Neurodegenerative diseases
Peripheral proteinopathies
Amyloid β
Tau
α-Synuclein
TDP-43
url https://doi.org/10.1186/s40035-024-00461-6
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AT junchen peripheralproteinopathyinneurodegenerativediseases
AT zhixu peripheralproteinopathyinneurodegenerativediseases
AT keqiangye peripheralproteinopathyinneurodegenerativediseases
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