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@ARTICLE{Brs:165354,
author = {Brás, Inês C and Khani, Mohammad H and Vasili, Eftychia
and Möbius, Wiebke and Riedel, Dietmar and Parfentev, Iwan
and Gerhardt, Ellen and Fahlbusch, Christiane and Urlaub,
Henning and Zweckstetter, Markus and Gollisch, Tim and
Outeiro, Tiago F},
title = {{M}olecular {M}echanisms {M}ediating the {T}ransfer of
{D}isease-{A}ssociated {P}roteins and {E}ffects on
{N}euronal {A}ctivity.},
journal = {Journal of Parkinson's Disease},
volume = {12},
number = {8},
issn = {1877-7171},
address = {Amsterdam},
publisher = {IOS Press},
reportid = {DZNE-2022-01631},
pages = {2397 - 2422},
year = {2022},
abstract = {Various cellular pathways have been implicated in the
transfer of disease-related proteins between cells,
contributing to disease progression and neurodegeneration.
However, the overall effects of protein transfer are still
unclear.Here, we performed a systematic comparison of basic
molecular mechanisms involved in the release of
alpha-synuclein, Tau, and huntingtin, and evaluated
functional effects upon internalization by receiving
cells.Evaluation of protein release to the extracellular
space in a free form and in extracellular vesicles using an
optimized ultracentrifugation protocol. The extracellular
effects of the proteins and extracellular vesicles in
primary neuronal cultures were assessed using multi-channel
electrophysiological recordings combined with a customized
spike sorting framework.We demonstrate cells differentially
release free-forms of each protein to the extracellular
space. Importantly, neuronal activity is distinctly
modulated upon protein internalization in primary cortical
cultures. In addition, these disease-related proteins also
occur in extracellular vesicles, and are enriched in
ectosomes. Internalization of ectosomes and exosomes by
primary microglial or astrocytic cells elicits the
production of pro-inflammatory cytokines, and modifies
spontaneous electrical activity in neurons.Overall, our
study demonstrates that released proteins can have
detrimental effects for surrounding cells, and suggests
protein release pathways may be exploited as therapeutic
targets in different neurodegenerative diseases.},
keywords = {Humans / Parkinson Disease: metabolism / Neurons:
metabolism / Exosomes: metabolism / Protein Transport /
Alpha-synuclein (Other) / Tau (Other) / extracellular
vesicles (Other) / huntingtin (Other) / neuronal function
(Other)},
cin = {AG Fischer / AG Zweckstetter},
ddc = {610},
cid = {I:(DE-2719)1410002 / I:(DE-2719)1410001},
pnm = {352 - Disease Mechanisms (POF4-352)},
pid = {G:(DE-HGF)POF4-352},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:36278361},
doi = {10.3233/JPD-223516},
url = {https://pub.dzne.de/record/165354},
}