TY - JOUR
AU - Zhang, Shuyu
AU - Dauer, Karina
AU - Strohäker, Timo
AU - Tatenhorst, Lars
AU - Caldi Gomes, Lucas
AU - Mayer, Simon
AU - Jung, Byung Chul
AU - Kim, Woojin S
AU - Lee, Seung-Jae
AU - Becker, Stefan
AU - Liesche-Starnecker, Friederike
AU - Zweckstetter, Markus
AU - Lingor, Paul
TI - Alpha-synuclein fibrils amplified from multiple system atrophy and Parkinson's disease patient brain spread after intracerebral injection into mouse brain.
JO - Brain pathology
VL - 33
IS - 5
SN - 1015-6305
CY - Oxford
PB - Wiley-Blackwell
M1 - DZNE-2023-00843
SP - e13196
PY - 2023
AB - Parkinson's disease (PD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB) are neurodegenerative disorders with alpha-synuclein (α-syn) aggregation pathology. Different strains of α-syn with unique properties are suggested to cause distinct clinical and pathological manifestations resulting in PD, MSA, or DLB. To study individual α-syn spreading patterns, we injected α-syn fibrils amplified from brain homogenates of two MSA patients and two PD patients into the brains of C57BI6/J mice. Antibody staining against pS129-α-syn showed that α-syn fibrils amplified from the brain homogenates of the four different patients caused different levels of α-syn spreading. The strongest α-syn pathology was triggered by α-syn fibrils of one of the two MSA patients, followed by comparable pS129-α-syn induction by the second MSA and one PD patient material. Histological analysis using an antibody against Iba1 further showed that the formation of pS129-α-syn is associated with increased microglia activation. In contrast, no differences in dopaminergic neuron numbers or co-localization of α-syn in oligodendrocytes were observed between the different groups. Our data support the spreading of α-syn pathology in MSA, while at the same time pointing to spreading heterogeneity between different patients potentially driven by individual patient immanent factors.
KW - Animals
KW - Mice
KW - alpha-Synuclein: metabolism
KW - Parkinson Disease: pathology
KW - Multiple System Atrophy: pathology
KW - Brain: pathology
KW - Synucleinopathies: pathology
KW - Antibodies
KW - Snca protein, mouse (NLM Chemicals)
KW - Parkinson's disease (Other)
KW - alpha-synuclein (Other)
KW - microglia (Other)
KW - multiple system atrophy (Other)
KW - patient-derived fibrils (Other)
KW - alpha-Synuclein (NLM Chemicals)
KW - Antibodies (NLM Chemicals)
LB - PUB:(DE-HGF)16
C2 - pmc:PMC10467043
C6 - pmid:37485772
DO - DOI:10.1111/bpa.13196
UR - https://pub.dzne.de/record/263624
ER -