α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies
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α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies. / Schmitz, Matthias; Candelise, Niccolò; Canaslan, Sezgi; Altmeppen, Hermann C; Matschke, Jakob; Glatzel, Markus; Younas, Neelam; Zafar, Saima; Hermann, Peter; Zerr, Inga.
In: TRANSL NEURODEGENER, Vol. 12, No. 1, 14.03.2023, p. 12.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - α-Synuclein conformers reveal link to clinical heterogeneity of α-synucleinopathies
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
N1 - © 2023. The Author(s).
PY - 2023/3/14
Y1 - 2023/3/14
N2 - 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.
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 - Synucleinopathies/diagnosis
KW - Parkinson Disease/diagnosis
KW - Lewy Bodies/pathology
KW - Multiple System Atrophy/diagnosis
U2 - 10.1186/s40035-023-00342-4
DO - 10.1186/s40035-023-00342-4
M3 - SCORING: Review article
C2 - 36915212
VL - 12
SP - 12
JO - TRANSL NEURODEGENER
JF - TRANSL NEURODEGENER
SN - 2047-9158
IS - 1
ER -
