Autophagy in α-Synucleinopathies—An Overstrained System

Alpha-synucleinopathies comprise progressive neurodegenerative diseases, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). They all exhibit the same pathological hallmark, which is the formation of α-synuclein positive deposits in neuronal or gli...

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Autores principales:	Lisa Fellner, Elisa Gabassi, Johannes Haybaeck, Frank Edenhofer
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	alpha-synuclein Parkinson’s disease multiple system atrophy autophagy neurons oligodendroglia Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/9e0458cf8cf94b649567d2a377387334
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id	oai:doaj.org-article:9e0458cf8cf94b649567d2a377387334
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spelling	oai:doaj.org-article:9e0458cf8cf94b649567d2a3773873342021-11-25T17:11:56ZAutophagy in α-Synucleinopathies—An Overstrained System10.3390/cells101131432073-4409https://doaj.org/article/9e0458cf8cf94b649567d2a3773873342021-11-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/3143https://doaj.org/toc/2073-4409Alpha-synucleinopathies comprise progressive neurodegenerative diseases, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). They all exhibit the same pathological hallmark, which is the formation of α-synuclein positive deposits in neuronal or glial cells. The aggregation of α-synuclein in the cell body of neurons, giving rise to the so-called Lewy bodies (LBs), is the major characteristic for PD and DLB, whereas the accumulation of α-synuclein in oligodendroglial cells, so-called glial cytoplasmic inclusions (GCIs), is the hallmark for MSA. The mechanisms involved in the intracytoplasmic inclusion formation in neuronal and oligodendroglial cells are not fully understood to date. A possible mechanism could be an impaired autophagic machinery that cannot cope with the high intracellular amount of α-synuclein. In fact, different studies showed that reduced autophagy is involved in α-synuclein aggregation. Furthermore, altered levels of different autophagy markers were reported in PD, DLB, and MSA brains. To date, the trigger point in disease initiation is not entirely clear; that is, whether autophagy dysfunction alone suffices to increase α-synuclein or whether α-synuclein is the pathogenic driver. In the current review, we discuss the involvement of defective autophagy machinery in the formation of α-synuclein aggregates, propagation of α-synuclein, and the resulting neurodegenerative processes in α-synucleinopathies.Lisa FellnerElisa GabassiJohannes HaybaeckFrank EdenhoferMDPI AGarticlealpha-synucleinParkinson’s diseasemultiple system atrophyautophagyneuronsoligodendrogliaBiology (General)QH301-705.5ENCells, Vol 10, Iss 3143, p 3143 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	alpha-synuclein Parkinson’s disease multiple system atrophy autophagy neurons oligodendroglia Biology (General) QH301-705.5
spellingShingle	alpha-synuclein Parkinson’s disease multiple system atrophy autophagy neurons oligodendroglia Biology (General) QH301-705.5 Lisa Fellner Elisa Gabassi Johannes Haybaeck Frank Edenhofer Autophagy in α-Synucleinopathies—An Overstrained System
description	Alpha-synucleinopathies comprise progressive neurodegenerative diseases, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). They all exhibit the same pathological hallmark, which is the formation of α-synuclein positive deposits in neuronal or glial cells. The aggregation of α-synuclein in the cell body of neurons, giving rise to the so-called Lewy bodies (LBs), is the major characteristic for PD and DLB, whereas the accumulation of α-synuclein in oligodendroglial cells, so-called glial cytoplasmic inclusions (GCIs), is the hallmark for MSA. The mechanisms involved in the intracytoplasmic inclusion formation in neuronal and oligodendroglial cells are not fully understood to date. A possible mechanism could be an impaired autophagic machinery that cannot cope with the high intracellular amount of α-synuclein. In fact, different studies showed that reduced autophagy is involved in α-synuclein aggregation. Furthermore, altered levels of different autophagy markers were reported in PD, DLB, and MSA brains. To date, the trigger point in disease initiation is not entirely clear; that is, whether autophagy dysfunction alone suffices to increase α-synuclein or whether α-synuclein is the pathogenic driver. In the current review, we discuss the involvement of defective autophagy machinery in the formation of α-synuclein aggregates, propagation of α-synuclein, and the resulting neurodegenerative processes in α-synucleinopathies.
format	article
author	Lisa Fellner Elisa Gabassi Johannes Haybaeck Frank Edenhofer
author_facet	Lisa Fellner Elisa Gabassi Johannes Haybaeck Frank Edenhofer
author_sort	Lisa Fellner
title	Autophagy in α-Synucleinopathies—An Overstrained System
title_short	Autophagy in α-Synucleinopathies—An Overstrained System
title_full	Autophagy in α-Synucleinopathies—An Overstrained System
title_fullStr	Autophagy in α-Synucleinopathies—An Overstrained System
title_full_unstemmed	Autophagy in α-Synucleinopathies—An Overstrained System
title_sort	autophagy in α-synucleinopathies—an overstrained system
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/9e0458cf8cf94b649567d2a377387334
work_keys_str_mv	AT lisafellner autophagyinasynucleinopathiesanoverstrainedsystem AT elisagabassi autophagyinasynucleinopathiesanoverstrainedsystem AT johanneshaybaeck autophagyinasynucleinopathiesanoverstrainedsystem AT frankedenhofer autophagyinasynucleinopathiesanoverstrainedsystem
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Autophagy in α-Synucleinopathies—An Overstrained System

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