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Review
. 2018 Aug 17:12:574.
doi: 10.3389/fnins.2018.00574. eCollection 2018.

Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis

Deborah Ferrara  1 , Laura Pasetto  2 , Valentina Bonetto  2 , Manuela Basso  1
Affiliations
Review

Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis

Deborah Ferrara et al. Front Neurosci. .

Abstract

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.

Keywords: amyotrophic lateral sclerosis; biomarkers; extracellular vesicles; prion-like behavior; therapy.

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Figures

FIGURE 1
FIGURE 1
EVs in ALS pathology. Motor neurons, astrocytes, microglia, lymphocytes, and muscles interplay and contribute to ALS pathogenesis. Mutant proteins implicated in ALS, such as mutant SOD1, TDP-43, FUS, and DPRs derived from expanded C9orf72, were detected in EVs and suggest EVs as means of disease spreading.
See this image and copyright information in PMC

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