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@ARTICLE{Lohmann:141488,
author = {Lohmann, Stephanie and Bernis, Maria E and Babila, Julius
Tachu and Ziemski, Alexandra and Grigoletto, Jessica and
Tamgüney, Gültekin},
title = {{O}ral and intravenous transmission of α-synuclein fibrils
to mice.},
journal = {Acta neuropathologica},
volume = {138},
number = {4},
issn = {0001-6322},
address = {Heidelberg},
publisher = {Springer},
reportid = {DZNE-2020-07812},
pages = {515-533},
year = {2019},
abstract = {Parkinson's disease and related disorders are
neuropathologically characterized by cellular deposits of
misfolded and aggregated α-synuclein in the CNS.
Disease-associated α-synuclein adopts a conformation that
causes it to form oligomers and fibrils, which have reduced
solubility, become hyperphosphorylated, and contribute to
the spatiotemporal spreading of pathology in the CNS. The
infectious properties of disease-associated α-synuclein,
e.g., by which peripheral route and with which efficiency it
can be transmitted, are not fully understood. Here, we
investigated the potential of α-synuclein fibrils to induce
neurological disease in TgM83+/- mice expressing the A53T
mutant of human α-synuclein after oral or intravenous
challenge and compared it to intraperitoneal and
intracerebral challenge. Oral challenge with 50 µg of
α-synuclein fibrils caused neurological disease in two out
of eight mice in 220 days and 350 days, and challenge with
500 µg in four out of eight mice in 384 ± 131 days,
respectively. Intravenous challenge with 50 µg of
α-synuclein fibrils led to disease in 208 ± 20 days
in 10 out of 10 mice and was in duration comparable to
intraperitoneal challenge with 50 µg of α-synuclein
fibrils, which caused disease in 10 out of 10 mice in
202 ± 35 days. Ten out of 10 mice that were each
intracerebrally challenged with 10 µg or 50 µg of
α-synuclein fibrils developed disease in
156 ± 20 days and 133 ± 4 days, respectively.
The CNS of diseased mice displayed aggregates of
sarkosyl-insoluble and phosphorylated α-synuclein, which
colocalized with ubiquitin and p62 and were accompanied by
gliosis indicative of neuroinflammation. 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 are important,
because they show that α-synuclein fibrils can neuroinvade
the CNS after a single oral or intravenous challenge and
cause neuropathology and disease.},
keywords = {Administration, Intravenous / Administration, Oral /
Animals / Brain: drug effects / Brain: pathology / Disease
Models, Animal / Mice / Phosphorylation / Synucleinopathies:
chemically induced / Synucleinopathies: pathology /
alpha-Synuclein: administration $\&$ dosage /
alpha-Synuclein: metabolism},
cin = {AG Tamgüney 2},
ddc = {610},
cid = {I:(DE-2719)1013022},
pnm = {342 - Disease Mechanisms and Model Systems (POF3-342)},
pid = {G:(DE-HGF)POF3-342},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:31230104},
pmc = {pmc:PMC6778172},
doi = {10.1007/s00401-019-02037-5},
url = {https://pub.dzne.de/record/141488},
}