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14 Results Found

  • Article
  • Open Access
16 Citations
10,572 Views
17 Pages

Oligodendrocytes Prune Axons Containing α-Synuclein Aggregates In Vivo: Lewy Neurites as Precursors of Glial Cytoplasmic Inclusions in Multiple System Atrophy?

  • Francesco De Nuccio,
  • Marianna Kashyrina,
  • Francesca Serinelli,
  • Florent Laferrière and ,
  • Dario Domenico Lofrumento,
  • Francesca De Giorgi and
  • François Ichas
  • + 2 authors+ 0 author

1 February 2023

α-Synucleinopathies are spreading neurodegenerative disorders characterized by the intracellular accumulation of insoluble aggregates populated by α-Synuclein (α-Syn) fibrils. In Parkinson’s disease (PD) and dementia with Lewy...

(This article belongs to the Section Molecular Medicine)
  • Article
  • Open Access
35 Citations
6,571 Views
15 Pages

Pathological Relevance of Post-Translationally Modified Alpha-Synuclein (pSer87, pSer129, nTyr39) in Idiopathic Parkinson’s Disease and Multiple System Atrophy

  • Berkiye Sonustun,
  • Melek Firat Altay,
  • Catherine Strand,
  • Kirsten Ebanks and ,
  • Geshanthi Hondhamuni,
  • Thomas T. Warner,
  • Hilal A. Lashuel and
  • Rina Bandopadhyay
  • + 3 authors+ 0 author

6 March 2022

Aggregated alpha-synuclein (α-synuclein) is the main component of Lewy bodies (LBs), Lewy neurites (LNs), and glial cytoplasmic inclusions (GCIs), which are pathological hallmarks of idiopathic Parkinson’s disease (IPD) and multiple syste...

(This article belongs to the Special Issue Parkinson’s Disease (PD): Molecular Mechanisms and Novel Treatment Strategies)
  • Review
  • Open Access
82 Citations
24,111 Views
35 Pages

17 March 2009

Parkinson’s disease (PD) is the second most common progressive neurodegenerative disorder after Alzheimer's disease (AD) and represents a large health burden to society. Genetic and oxidative risk factors have been proposed as possible causes, but th...

(This article belongs to the Special Issue Advances in Molecular Neuropathology)
  • Review
  • Open Access
53 Citations
10,304 Views
53 Pages

Neurons and Glia Interplay in α-Synucleinopathies

  • Panagiota Mavroeidi and
  • Maria Xilouri

Accumulation of the neuronal presynaptic protein alpha-synuclein within proteinaceous inclusions represents the key histophathological hallmark of a spectrum of neurodegenerative disorders, referred to by the umbrella term a-synucleinopathies. Even t...

(This article belongs to the Section Molecular Neurobiology)
  • Review
  • Open Access
78 Citations
10,344 Views
24 Pages

Autophagy in Synucleinopathy: The Overwhelmed and Defective Machinery

  • Marie-Laure Arotcarena,
  • Margaux Teil and
  • Benjamin Dehay

9 June 2019

Alpha-synuclein positive-intracytoplasmic inclusions are the common denominators of the synucleinopathies present as Lewy bodies in Parkinson’s disease, dementia with Lewy bodies, or glial cytoplasmic inclusions in multiple system atrophy. Thes...

(This article belongs to the Special Issue Autophagy in Neurodegenerative Diseases)
  • Review
  • Open Access
29 Citations
6,723 Views
18 Pages

Autophagy in α-Synucleinopathies—An Overstrained System

  • Lisa Fellner,
  • Elisa Gabassi,
  • Johannes Haybaeck and
  • Frank Edenhofer

12 November 2021

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 o...

(This article belongs to the Special Issue 10th Anniversary of Cells—Advances in Autophagy)
  • Review
  • Open Access
1 Citations
1,152 Views
19 Pages

MicroRNA as Potential Biomarkers and Their Pathogenesis in Multiple System Atrophy

  • Ming-Che Kuo,
  • Shao-Ying Cheng,
  • Meng-Ling Chen and
  • Ruey-Meei Wu

15 February 2026

Multiple system atrophy (MSA) is a rare, rapidly progressive neurodegenerative disorder characterized by autonomic dysfunction, Parkinsonism, and cerebellar ataxia. While the pathological hallmark of MSA is the accumulation of α-synuclein in ol...

(This article belongs to the Special Issue MicroRNAs as Biomarkers and Therapeutic Targets in Human Diseases)
  • Article
  • Open Access
7 Citations
3,716 Views
17 Pages

Phase Separation of SARS-CoV-2 Nucleocapsid Protein with TDP-43 Is Dependent on C-Terminus Domains

  • Michael J. Strong,
  • Crystal McLellan,
  • Brianna Kaplanis,
  • Cristian A. Droppelmann and
  • Murray Junop

12 August 2024

The SARS-CoV-2 nucleocapsid protein (N protein) is critical in viral replication by undergoing liquid–liquid phase separation to seed the formation of a ribonucleoprotein (RNP) complex to drive viral genomic RNA (gRNA) translation and in suppre...

(This article belongs to the Special Issue RNA Virus Mechanisms: From Molecular Insights to Therapeutic Strategies)
  • Feature Paper
  • Article
  • Open Access
18 Citations
7,587 Views
22 Pages

Overexpression of α-Synuclein by Oligodendrocytes in Transgenic Mice Does Not Recapitulate the Fibrillar Aggregation Seen in Multiple System Atrophy

  • Florent Laferrière,
  • Xin He,
  • Federica Zinghirino,
  • Evelyne Doudnikoff and ,
  • Emilie Faggiani,
  • Wassilios G. Meissner,
  • Erwan Bezard,
  • Francesca De Giorgi and
  • François Ichas
  • + 4 authors+ 0 author

29 October 2020

The synucleinopathy underlying multiple system atrophy (MSA) is characterized by the presence of abundant amyloid inclusions containing fibrillar α-synuclein (α-syn) aggregates in the brains of the patients and is associated with an exten...

(This article belongs to the Special Issue Key Signalling Molecules in Aging and Neurodegeneration)
  • Review
  • Open Access
91 Citations
9,323 Views
14 Pages

α-Synuclein is a protein with a molecular weight of 14.5 kDa and consists of 140 amino acids encoded by the SNCA gene. Missense mutations and gene duplications in the SNCA gene cause hereditary Parkinson’s disease. Highly phosphorylated a...

(This article belongs to the Special Issue Protein Kinases and Neurodegenerative Diseases)
  • Review
  • Open Access
24 Citations
8,665 Views
17 Pages

20 November 2024

The abnormal deposition and intercellular propagation of disease-specific protein play a central role in the pathogenesis of many neurodegenerative disorders. Recent studies share the common observation that the formation of protein oligomers and sub...

(This article belongs to the Special Issue Neurological Disorders: Molecular Pathology and Therapeutic Approaches)
  • Review
  • Open Access
15 Citations
6,199 Views
17 Pages

Is Multiple System Atrophy a Prion-like Disorder?

  • Kurt A. Jellinger,
  • Gregor K. Wenning and
  • Nadia Stefanova

18 September 2021

Multiple system atrophy (MSA) is a rapidly progressive, fatal neurodegenerative disease of uncertain aetiology that belongs to the family of α-synucleinopathies. It clinically presents with parkinsonism, cerebellar, autonomic, and motor impairment in...

(This article belongs to the Collection Feature Papers in Molecular Pathology, Diagnostics, and Therapeutics)
  • Review
  • Open Access
34 Citations
11,776 Views
18 Pages

Multiple system atrophy (MSA) is a fatal, rapidly progressing neurodegenerative disease of uncertain etiology, clinically characterized by various combinations of Levodopa unresponsive parkinsonism, cerebellar, autonomic and motor dysfunctions. The m...

(This article belongs to the Section Neurobiology and Clinical Neuroscience)
  • Article
  • Open Access
5 Citations
3,612 Views
59 Pages

The ultrastructural organization of the nuclei of the tegmental region in juvenile chum salmon (Oncorhynchus keta) was examined using transmission electron microscopy (TEM). The dorsal tegmental nuclei (DTN), the nucleus of fasciculus longitudinalis ...

(This article belongs to the Special Issue Molecular Research on Brain Injury)