Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is the most common motor neuron disease in adults and primarily targets upper and lower motor neurons. The progression of the disease is mostly mediated by altered intercellular communication in the spinal cord between neurons and glial cells. One of the possible...

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Main Authors: Deborah Ferrara, Laura Pasetto, Valentina Bonetto, Manuela Basso
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-08-01
Series:Frontiers in Neuroscience
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fnins.2018.00574/full
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author Deborah Ferrara
Laura Pasetto
Valentina Bonetto
Manuela Basso
author_facet Deborah Ferrara
Laura Pasetto
Valentina Bonetto
Manuela Basso
author_sort Deborah Ferrara
collection DOAJ
description Amyotrophic Lateral Sclerosis (ALS) is the most common motor neuron disease in adults and primarily targets upper and lower motor neurons. The progression of the disease is mostly mediated by altered intercellular communication in the spinal cord between neurons and glial cells. One of the possible ways by which intercellular communication occurs is through extracellular vesicles (EVs) that are responsible for the horizontal transfer of proteins and RNAs to recipient cells. EVs are nanoparticles released by the plasma membrane and this review will describe all evidence connecting ALS, intercellular miscommunication and EVs. We mainly focus on mutant proteins causing ALS and their accumulation in EVs, along with the propensity of mutant proteins to misfold and propagate through EVs in prion-like behavior. EVs are a promising source of biomarkers and the state of the art in ALS will be discussed along with the gaps and challenges still present in this blooming field of investigation.
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spelling doaj.art-8d825332f0ef4f67979306d7226a11ee2022-12-21T19:42:26ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2018-08-011210.3389/fnins.2018.00574408165Role of Extracellular Vesicles in Amyotrophic Lateral SclerosisDeborah Ferrara0Laura Pasetto1Valentina Bonetto2Manuela Basso3Laboratory of Transcriptional Neurobiology, Centre for Integrative Biology (CIBIO), University of Trento, Trento, ItalyDepartment of Molecular Biochemistry and Pharmacology, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Milan, ItalyDepartment of Molecular Biochemistry and Pharmacology, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Milan, ItalyLaboratory of Transcriptional Neurobiology, Centre for Integrative Biology (CIBIO), University of Trento, Trento, ItalyAmyotrophic Lateral Sclerosis (ALS) is the most common motor neuron disease in adults and primarily targets upper and lower motor neurons. The progression of the disease is mostly mediated by altered intercellular communication in the spinal cord between neurons and glial cells. One of the possible ways by which intercellular communication occurs is through extracellular vesicles (EVs) that are responsible for the horizontal transfer of proteins and RNAs to recipient cells. EVs are nanoparticles released by the plasma membrane and this review will describe all evidence connecting ALS, intercellular miscommunication and EVs. We mainly focus on mutant proteins causing ALS and their accumulation in EVs, along with the propensity of mutant proteins to misfold and propagate through EVs in prion-like behavior. EVs are a promising source of biomarkers and the state of the art in ALS will be discussed along with the gaps and challenges still present in this blooming field of investigation.https://www.frontiersin.org/article/10.3389/fnins.2018.00574/fullextracellular vesiclesamyotrophic lateral sclerosisbiomarkersprion-like behaviortherapy
spellingShingle Deborah Ferrara
Laura Pasetto
Valentina Bonetto
Manuela Basso
Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
Frontiers in Neuroscience
extracellular vesicles
amyotrophic lateral sclerosis
biomarkers
prion-like behavior
therapy
title Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
title_full Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
title_fullStr Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
title_full_unstemmed Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
title_short Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis
title_sort role of extracellular vesicles in amyotrophic lateral sclerosis
topic extracellular vesicles
amyotrophic lateral sclerosis
biomarkers
prion-like behavior
therapy
url https://www.frontiersin.org/article/10.3389/fnins.2018.00574/full
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AT manuelabasso roleofextracellularvesiclesinamyotrophiclateralsclerosis