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Title: Extracellular Vesicles Physiological Role and the Particular Case of Disease-Spreading Mechanisms in Polyglutamine Diseases
Authors: Moreira, Ricardo 
Mendonça, Liliana Simões 
Almeida, Luís Pereira de 
Keywords: extracellular vesicles;; disease spreading;; neurodegenerative diseases;; polyglutamine diseases;; vehicle;; biomarker
Issue Date: 13-Nov-2021
Publisher: MDPI
Project: CENTRO-01- 0145-FEDER-000008 
PhD Scholarship 2020.04751.BD 
National Ataxia Foundation 
French Muscular Dystrophy Association (AFM-Téléthon) Trampoline Grant #20126 
Richard Chin and Lily Lock Machado–Joseph Disease Research Fund 
JPND project SynSpread 
Serial title, monograph or event: International Journal of Molecular Sciences
Volume: 22
Issue: 22
Abstract: Recent research demonstrated pathological spreading of the disease-causing proteins from one focal point across other brain regions for some neurodegenerative diseases, such as Parkinson's and Alzheimer's disease. Spreading mediated by extracellular vesicles is one of the proposed disease-spreading mechanisms. Extracellular vesicles are cell membrane-derived vesicles, used by cells for cell-to-cell communication and excretion of toxic components. Importantly, extracellular vesicles carrying pathological molecules, when internalized by "healthy" cells, may trigger pathological pathways and, consequently, promote disease spreading to neighboring cells. Polyglutamine diseases are a group of genetic neurodegenerative disorders characterized by the accumulation of mutant misfolded proteins carrying an expanded tract of glutamines, including Huntington's and Machado-Joseph disease. The pathological spread of the misfolded proteins or the corresponding mutant mRNA has been explored. The understanding of the disease-spreading mechanism that plays a key role in the pathology progression of these diseases can result in the development of effective therapeutic approaches to stop disease progression, arresting the spread of the toxic components and disease aggravation. Therefore, the present review's main focus is the disease-spreading mechanisms with emphasis on polyglutamine diseases and the putative role played by extracellular vesicles in this process.
ISSN: 1422-0067
DOI: 10.3390/ijms222212288
Rights: openAccess
Appears in Collections:I&D CNC - Artigos em Revistas Internacionais
I&D CIBB - Artigos em Revistas Internacionais
FFUC- Artigos em Revistas Internacionais

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