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@ARTICLE{VanDenBerge:872758,
author = {Van Den Berge, Nathalie and Ferreira, Nelson and Gram,
Hjalte and Mikkelsen, Trine Werenberg and Alstrup, Aage
Kristian Olsen and Casadei, Nicolas and Tsung-Pin, Pai and
Riess, Olaf and Nyengaard, Jens Randel and Tamgüney,
Gültekin and Jensen, Poul Henning and Borghammer, Per},
title = {{E}vidence for bidirectional and trans-synaptic
parasympathetic and sympathetic propagation of
alpha-synuclein in rats},
journal = {Acta neuropathologica},
volume = {138},
number = {4},
issn = {1432-0533},
address = {Heidelberg},
publisher = {Springer},
reportid = {FZJ-2020-00236},
pages = {535 - 550},
year = {2019},
abstract = {The conversion of endogenous alpha-synuclein (asyn) to
pathological asyn-enriched aggregates is a hallmark of
Parkinson’s disease (PD). These inclusions can be detected
in the central and enteric nervous system (ENS). Moreover,
gastrointestinal symptoms can appear up to 20 years before
the diagnosis of PD. The dual-hit hypothesis posits that
pathological asyn aggre-gation starts in the ENS, and
retrogradely spreads to the brain. In this study, we tested
this hypothesis by directly injecting preformed asyn fibrils
into the duodenum wall of wild-type rats and transgenic rats
with excess levels of human asyn. We provide a meticulous
characterization of the bacterial artificial chromosome
(BAC) transgenic rat model with respect to ini-tial
propagation of pathological asyn along the parasympathetic
and sympathetic pathways to the brainstem, by performing
immunohistochemistry at early time points post-injection.
Induced pathology was observed in all key structures along
the sympathetic and parasympathetic pathways (ENS, autonomic
ganglia, intermediolateral nucleus of the spinal cord (IML),
heart, dorsal motor nucleus of the vagus, and locus
coeruleus (LC)) and persisted for at least 4 months
post-injection. In contrast, asyn propagation was not
detected in wild-type rats, nor in vehicle-injected BAC
rats. The presence of pathology in the IML, LC, and heart
indicate trans-synaptic spread of the pathology.
Additionally, the observed asyn inclusions in the stomach
and heart may indicate secondary anterograde propagation
after initial retrograde spreading. In summary,
trans-synaptic propagation of asyn in the BAC rat model is
fully compatible with the “body-first hypothesis” of PD
etiopatho-genesis. To our knowledge, this is the first
animal model evidence of asyn propagation to the heart, and
the first indication of bidirectional asyn propagation via
the vagus nerve, i.e., duodenum-to-brainstem-to-stomach. The
BAC rat model could be very valuable for detailed
mechanistic studies of the dual-hit hypothesis, and for
studies of disease modifying therapies targeting early
pathology in the gastrointestinal tract.},
cin = {ICS-6},
ddc = {610},
cid = {I:(DE-Juel1)ICS-6-20110106},
pnm = {553 - Physical Basis of Diseases (POF3-553)},
pid = {G:(DE-HGF)POF3-553},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:31254094},
UT = {WOS:000488931000004},
doi = {10.1007/s00401-019-02040-w},
url = {https://juser.fz-juelich.de/record/872758},
}