Abstract
Huntington’s disease (HD) is a rare hereditary autosomal dominant neurodegenerative disorder, which is caused by expression of mutant huntingtin protein (mHTT) with an abnormal number of glutamine repeats in its N terminus, and characterized by intracellular mHTT aggregates (inclusions) in the brain. Exosomes are small extracellular vesicles that are secreted generally by all cell types and can be isolated from almost all body fluids such as blood, urine, saliva, and cerebrospinal fluid. Exosomes may participate in the spreading of toxic misfolded proteins across the central nervous system in neurodegenerative diseases. In HD, such propagation of mHTT was observed both in vitro and in vivo. On the other hand, exosomes might carry molecules with neuroprotective effects. In addition, due to their capability to cross blood-brain barrier, exosomes hold great potential as sources of biomarkers available from periphery or carriers of therapeutics into the central nervous system. In this review, we discuss the emerging roles of exosomes in HD pathogenesis, diagnosis, and therapy.
Highlights
Extracellular vesicles (EVs) are phospholipid bilayer membrane enveloped particles released from cells into extracellular environments and body fluids
Exosome biogenesis depends on on endosomal sorting complexes required for transport (ESCRTs), which recognize thethe endosomal sorting complexes required for transport (ESCRTs), which recognize ubiqubiquitinated proteins
On the other microvesicles arise by outward budding of cytoplasmic membrane and apoptotic bodies are formed hand, microvesicles arise by outward budding of cytoplasmic membrane and apoptotic bodies are during programmed cell death; (b) Exosomes contain proteins, RNAs, lipids and small molecules formed during programmed death; (B) Exosomes contain proteins, RNAs, lipids and small of the source cell
Summary
Extracellular vesicles (EVs) are phospholipid bilayer membrane enveloped particles released from cells into extracellular environments and body fluids. This review is focused mainly on exosomes, as they are the most studied vesicle subtype in human diseases, including. Exosomes play an important role in cell-cell communication and intercellular signaling by transferring molecules into recipient cells [5,6,7]. Due to their roles in the physiological and pathological conditions in the brain, their ability to cross the blood-brain barrier and their cargo, exosomes are a potential source of biomarkers or therapy carriers in neurodegenerative diseases (NDs)
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