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@PHDTHESIS{Lohmann:155350,
author = {Lohmann, Stephanie},
title = {{N}euroinvasion and cerebral ischemia as possible sources
for alpha-synuclein prions in {P}arkinson's disease},
school = {Rheinische Friedrich-Wilhelms-Universität Bonn},
type = {Dissertation},
reportid = {DZNE-2021-00617},
pages = {88 pages, 24 figures, 7 tables},
year = {2021},
note = {Dissertation, Rheinische Friedrich-Wilhelms-Universität
Bonn, 2021},
abstract = {In synucleinopathies such as Parkinson's disease misfolding
of a-synuclein, normally a cellular and soluble protein,
leads to the accumulation of insoluble protein aggregates
and to central nervous system disease (CNS). Misfolded
a-synuclein acts as a seed, by recruiting native a-synuclein
and inducing its misfolding into insoluble a-synuclein
aggregates. Aggregated a-synuclein shows prion-like
characteristics and spreads via cell-to-cell transmission
throughout the central nervous system but also within the
periphery, ultimately causing neurological disease.Because
the spatiotemporal spreading of pathological a-synuclein
from the periphery to the CNS is not fully elucidated this
work investigated and compared the spreading of pathological
a-synuclein after intravenous or oral inoculation with that
after intracerebral or intraperitoneal inoculation of
TgM83+1- mice overexpressing the A53T mutant of human
a-synuclein with a-synuclein fibrils. In accordance with
previous studies, an infection rate of $100\%$ was observed
for intracerebrally and intraperitoneally injected mice.
Moreover, this study is the first to show that a single
intravenous injection of a-synuclein fibrils causes
synucleinopathy in $100\%$ of the challenged mice, and even
a single oral gavage with a-synuclein fibrils results in
$50\%$ of the challenged mice in neurological disease.
Diseased mice displayed aggregates of sarkosyl-insoluble and
phosphorylated a-synuclein, which colocalized with ubiquitin
and p62 and were accompanied by gliosis, indicative of
neuroinflammation, throughout the CNS. In contrast, none of
the control mice that were challenged with bovine serum
albumin via the same routes developed any neurological
disease or neuropathology. These findings show that
aggregated a-synuclein behaves like a prion causing
neuropathology and CNS disease, not only after intracerebral
or intraperitoneal challenge but also by neuroinvasion after
a single intravenous, or oral challenge.Since cerebral
ischemia increases the risk of developing Parkinson's
disease (PD) without being clear what the underlying
mechanism is, the second aim of this work was to study the
impact of ischemic stroke on a-synuclein aggregation, which
is known to cause PD. To investigate post-ischemic changes
to the CNS, focal cerebral ischemia was induced by middle
cerebral artery occlusion (MCAO) in transgenic mice
overexpressing the A53T mutant of human of a-synuclein.
lschemic mice displayed significant motor deficits and lass
of dopaminergic neurons in the substantia nigra at 360 days
after MCAO. This was caused by a significantly increased
amount of aggregated a-synuclein, which was accompanied by
neuroinflammation as indicated by astrogliosis and
microgliosis. In summary, cerebral ischemia induced a
synucleinopathy with lass of dopaminergic neurons in the
substantia nigra resulting in motor deficits in a mouse
model of PD, which may explain why cerebral ischemia
increases the risk of PD.},
cin = {AG Vorberg},
cid = {I:(DE-2719)1013004},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)11},
urn = {urn:nbn:de:hbz:5-63044},
url = {https://pub.dzne.de/record/155350},
}