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001     256605
005     20240112171657.0
024 7 _ |a 10.1186/s40035-023-00342-4
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024 7 _ |a 2047-9158
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024 7 _ |a 2096-6466
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037 _ _ |a DZNE-2023-00357
041 _ _ |a English
082 _ _ |a 570
100 1 _ |a Schmitz, Matthias
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245 _ _ |a α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies.
260 _ _ |a London
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|b Biomed Central
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520 _ _ |a 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.
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650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a alpha-Synuclein: genetics
|2 MeSH
650 _ 2 |a alpha-Synuclein: metabolism
|2 MeSH
650 _ 2 |a Synucleinopathies: diagnosis
|2 MeSH
650 _ 2 |a Synucleinopathies: genetics
|2 MeSH
650 _ 2 |a Synucleinopathies: metabolism
|2 MeSH
650 _ 2 |a Parkinson Disease: diagnosis
|2 MeSH
650 _ 2 |a Parkinson Disease: genetics
|2 MeSH
650 _ 2 |a Parkinson Disease: metabolism
|2 MeSH
650 _ 2 |a Lewy Bodies: pathology
|2 MeSH
650 _ 2 |a Multiple System Atrophy: diagnosis
|2 MeSH
650 _ 2 |a Multiple System Atrophy: genetics
|2 MeSH
650 _ 2 |a Multiple System Atrophy: pathology
|2 MeSH
650 _ 7 |a α-synuclein
|2 Other
650 _ 7 |a Protein strains
|2 Other
650 _ 7 |a RT-QuIC
|2 Other
650 _ 7 |a α-synuclein
|2 Other
650 _ 7 |a α-synucleinopathies
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650 _ 7 |a alpha-Synuclein
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650 _ 7 |a α-synucleinopathies
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700 1 _ |a Candelise, Niccolò
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700 1 _ |a Canaslan, Sezgi
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700 1 _ |a Altmeppen, Hermann C
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700 1 _ |a Matschke, Jakob
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700 1 _ |a Glatzel, Markus
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700 1 _ |a Younas, Neelam
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700 1 _ |a Zafar, Saima
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700 1 _ |a Hermann, Peter
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700 1 _ |a Zerr, Inga
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773 _ _ |a 10.1186/s40035-023-00342-4
|g Vol. 12, no. 1, p. 12
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|t Translational neurodegeneration
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