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.

Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis / Ferrara, Deborah; Pasetto, Laura; Bonetto, Valentina; Basso, Manuela. - In: FRONTIERS IN NEUROSCIENCE. - ISSN 1662-4548. - 12:(2018), p. 574. [10.3389/fnins.2018.00574]

Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis

Ferrara, Deborah;Basso, Manuela
2018

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.
Ferrara, Deborah; Pasetto, Laura; Bonetto, Valentina; Basso, Manuela
Role of Extracellular Vesicles in Amyotrophic Lateral Sclerosis / Ferrara, Deborah; Pasetto, Laura; Bonetto, Valentina; Basso, Manuela. - In: FRONTIERS IN NEUROSCIENCE. - ISSN 1662-4548. - 12:(2018), p. 574. [10.3389/fnins.2018.00574]
File in questo prodotto:
File Dimensione Formato  
fnins-12-00574.pdf

accesso aperto

Tipologia: Versione editoriale (Publisher’s layout)
Licenza: Creative commons
Dimensione 673.06 kB
Formato Adobe PDF
673.06 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11572/222714
Citazioni
  • ???jsp.display-item.citation.pmc??? 15
  • Scopus 25
  • ???jsp.display-item.citation.isi??? 22
social impact