Journal Article (Review Article)

DZNE-2023-00357

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies.

Schmitz, M. (First author)DZNE* ; Candelise, N.Extern* ; Canaslan, S.DZNE* ; Altmeppen, H. C. ; Matschke, J. ; Glatzel, M. ; Younas, N.DZNE* ; Zafar, S.DZNE* ; Hermann, P.DZNE* ; Zerr, I. (Last author)DZNE*

2023
Biomed Central London

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Please use a persistent id in citations: doi:10.1186/s40035-023-00342-4

Abstract: α-Synucleinopathies, such as Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy, are a class of neurodegenerative diseases exhibiting intracellular inclusions of misfolded α-synuclein (αSyn), referred to as Lewy bodies or oligodendroglial cytoplasmic inclusions (Papp-Lantos bodies). Even though the specific cellular distribution of aggregated αSyn differs in PD and DLB patients, both groups show a significant pathological overlap, raising the discussion of whether PD and DLB are the same or different diseases. Besides clinical investigation, we will focus in addition on methodologies, such as protein seeding assays (real-time quaking-induced conversion), to discriminate between different types of α-synucleinopathies. This approach relies on the seeding conversion properties of misfolded αSyn, supporting the hypothesis that different conformers of misfolded αSyn may occur in different types of α-synucleinopathies. Understanding the pathological processes influencing the disease progression and phenotype, provoked by different αSyn conformers, will be important for a personalized medical treatment in future.

Keyword(s): Humans (MeSH) ; alpha-Synuclein: genetics (MeSH) ; alpha-Synuclein: metabolism (MeSH) ; Synucleinopathies: diagnosis (MeSH) ; Synucleinopathies: genetics (MeSH) ; Synucleinopathies: metabolism (MeSH) ; Parkinson Disease: diagnosis (MeSH) ; Parkinson Disease: genetics (MeSH) ; Parkinson Disease: metabolism (MeSH) ; Lewy Bodies: pathology (MeSH) ; Multiple System Atrophy: diagnosis (MeSH) ; Multiple System Atrophy: genetics (MeSH) ; Multiple System Atrophy: pathology (MeSH) ; α-synuclein ; Protein strains ; RT-QuIC ; α-synuclein ; α-synucleinopathies ; alpha-Synuclein ; α-synucleinopathies

Note: CC BY

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Contributing Institute(s):

1. Translational Studies and Biomarkers (AG Zerr)
2. Ext UMG Zerr (Ext UMG Zerr)
3. Clinical Dementia Research Göttingen (Clinical Dementia Research Göttingen)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

Appears in the scientific report 2023

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Database coverage:
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