TY  - JOUR
AU  - Schmitz, Matthias
AU  - Candelise, Niccolò
AU  - Canaslan, Sezgi
AU  - Altmeppen, Hermann C
AU  - Matschke, Jakob
AU  - Glatzel, Markus
AU  - Younas, Neelam
AU  - Zafar, Saima
AU  - Hermann, Peter
AU  - Zerr, Inga
TI  - α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies.
JO  - Translational neurodegeneration
VL  - 12
IS  - 1
SN  - 2047-9158
CY  - London
PB  - Biomed Central
M1  - DZNE-2023-00357
SP  - 12
PY  - 2023
N1  - CC BY
AB  - 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.
KW  - Humans
KW  - alpha-Synuclein: genetics
KW  - alpha-Synuclein: metabolism
KW  - Synucleinopathies: diagnosis
KW  - Synucleinopathies: genetics
KW  - Synucleinopathies: metabolism
KW  - Parkinson Disease: diagnosis
KW  - Parkinson Disease: genetics
KW  - Parkinson Disease: metabolism
KW  - Lewy Bodies: pathology
KW  - Multiple System Atrophy: diagnosis
KW  - Multiple System Atrophy: genetics
KW  - Multiple System Atrophy: pathology
KW  - α-synuclein (Other)
KW  - Protein strains (Other)
KW  - RT-QuIC (Other)
KW  - α-synuclein (Other)
KW  - α-synucleinopathies (Other)
KW  - alpha-Synuclein (NLM Chemicals)
KW  - α-synucleinopathies (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:36915212
C2  - pmc:PMC10012698
DO  - DOI:10.1186/s40035-023-00342-4
UR  - https://pub.dzne.de/record/256605
ER  -